The CPL poli
By the policongenital and perinatal lung disorders (CPL) The Erasmus MC sees adults with congenital lung disease. This means that people who have developed lung disease, in pregnancy or in baby time, can be investigated and treated here.
The CPL-poli was set up in 2019 by lung doctor Dr Lieke Kamphuis and she does this together with her colleague lung doctor Dr Arnold Duinisveld and Dr Lidewij Visser. This policy is currently the only centre of expertise in the world that focuses on this group.
The doctors of this poli see many adults with congenital pulmonary diseases, such as bronchopulmonary dysplasia (hereinafter BPD) by the doctors also called lung damage due to premature birth. In addition to investigating and treating this group, the doctors are gathering information by seeing many people with (a suspicion of) this condition. This allows the doctors to get a good idea of what a condition means and how it can best be treated.
The policy aims to see as many people with lung damage as possible through premature birth, so that better research can be done. The aim is also to set up an international directive so that doctors worldwide can better treat congenital lung disease.
Furthermore, the doctors consider it all three important that there is room for the story of the patient and that the doctors can give the recognition and recognition required.
What diseases do they treat?
There are many different conditions that may already occur during pregnancy, but complications may also occur after pregnancy. Think of oxygen deficiency during childbirth or a congenital disorder occurring at a later age.
The CPL-poli includes the following conditions:
- Bronchopulmonary dysplasia (BPD);
- Congenital diaphragmatics (where BPD-like complaints can occur);
- Congenital oesophageal atresia (there are often infection problems);
- People with certain syndromes with lung diseases;
- People who have swallowed or inhaled around childbirth as a baby meconium (a baby's first stools), causing problems;
- People with an oxygen deficiency in childbirth and who have become disabled for example;
- Congenital cysts;
- Other rare diseases.
People may have known their whole lives that they have a particular condition. However, at a later age, something happens by chance on an X-ray or a CT scan. It may even happen that someone (appearingly) does not have any complaints, but that it is discovered by chance during an investigation in the hospital. It may also occur that at a later age people discover that there is a genetic defect in the family, which may lead to a later diagnosis.
How does lung development proceed in a normal situation?
The lungs develop during pregnancy while the baby receives its oxygen through the umbilical cord. In a normal situation, it looks like this. In the fourth week of pregnancy arches arise that later form the pharynx. Furthermore, the trachea and larynx form.
In the period after that, the trachea and the tissue from which the alveoli later develop. Two branches emerge from the trachea: One to the left lung and one to the right lung. On both sides they branch further into small tubes, also called the bronchi. After about three months the bronchi already contain some blood vessels.
From the 25th week of pregnancy the first coarse alveoli, also called sacculi, are formed. These have a thick wall with some blood vessels. From this grow the final alveoli that cause oxygen to be absorbed into the blood and waste is disposed of.
From about 24 weeks onwards, the lung cells are going to produce surfactant, which causes the alveoli to open properly after birth when inhaling and not collapse when exhaling. It is only after 28 weeks that the cells make enough work to keep the alveoli open.1 In addition, more and more alveoli are emerging during this period.
After birth, a baby has about 5% of the alveoli that he will eventually have during his life. The first two years are still very large, but the lungs are only mature after puberty.2
How does someone get to the CPL?
People can join the CPL-poli from the age of 17 by referring to their family doctor or a doctor from the hospital. A referral from a general practitioner or another doctor in the hospital is always required. If there are doubts about a referral or if people have problems with it, contact the policy via: CentrumCPL@erasmusmc.nl. Especially for people with such complaints and a history of early birth, a referral can lead to a correct diagnosis that allows more targeted treatment. It is certainly useful for doctors to ask early birth. The doctors of the policy are in the process of sharing knowledge about lung damage by early birth through continuing training and podcasts.
What is Bronchopulmonary Dysplasia?
BPD is a condition that can be caused by premature birth. The lungs are mainly less well developed and work less well. BPD can also give complaints at a later age such as shortness of breath and poor condition.3
In premature babies, there are a number of lung problems such as incomplete construction of the alveoli and blood vessels. Also there is not enough surfactant present, this is a substance that helps to leave the alveoli open. To tackle these problems, artificial ventilation has been used since the 1950s. In order to compensate for the lack of surfactant, a high pressure on ventilation has been used, along with a large volume of ventilation and a high concentration of oxygen. This causes damage to the airways, causing an inflammatory reaction and fibrosis of the tissue. In some children, this essentially creates an illness image called bronchopulmonary dysplasia.4
At BPD there is still a distinction between the classic and the new form which is later explained.
People who get the diagnosis BPD or lung damage due to early birth are often young and often do not want to feel like a patient. This group would like to know what they can do and stay in life despite their diagnosis. On the one hand, this group is a patient and on the other hand they want to be able to participate in everyday life.
How often does BPD occur?
In the Netherlands, approximately 2,000 infants are born alive a year with a gestational duration of less than 32 weeks. Of this group, about 75% have lung problems. It is therefore estimated that 60,000 Dutch with lung problems due to premature birth are. The exact number is difficult as people often don't know themselves and think their complaints are normal.5
What do people with lung damage notice from premature birth?
People with lung damage due to premature birth can often notice different things like:
- Respiratory complaints complaints such as wheezing
- Cough
- Shortness of breath (at exercise)
- Recurrent respiratory infections
- Fatigue
- Hepatic impairment
People may not experience complaints themselves, but they may notice, for example, that they are less able to exercise than others, that they are no longer able to come along well or that they are more tired. This is the form of lung damage you have since you were born. As a result, people are unaware of such complaints. Because they have their whole life (unknowingly) certain complaints, they often do not know otherwise and it is quite normal for these people. Many people have many years of complaints, without them knowing. On closer examination there is often more going on, but due to the above mentioned causes it is hardly noticed by patients and professionals.
It often happens that people are diagnosed with asthma or COPD, while they actually have lung damage due to their early birth. The complaints of this type are consistent with asthma and COPD. Since the consequences of lung damage due to premature birth at a later age are still quite unknown to both general practitioners and pulmonary doctors, a person can get a better diagnosis.
How is the diagnosis made?
At present, diagnosis is made in infants during the official 36th week of pregnancy in a pregnancy of 32 weeks, diagnosis is made after 28 days but before the 56th day after birth. Radiological changes, gestational duration ( 32 weeks) and oxygen use for at least 28 days are considered. Other causes are also excluded before diagnosis is made.6
There are three degrees: mild, moderate and severe. Based on the table below, oxygen requirements are considered based on gestational duration and a judgment on severity can be made.
| | < 32 weeks of pregnancy | >32 weeks of pregnancy |
| Time measurement | In the 36th week of pregnancy | >28 days, but <56 days after birth or discharge, which takes place first. |
| Oxygen treatment >21% for at least 28 days and: | ||
| Mild BPD | Breaths room air (with or without mustache 1 l/min) | Breaths room air (with or without mustache 1 l/min) |
| Moderately severe BPD | Oxygen requirement of <30% | Oxygen requirement of <30% |
| Severe BPD | Oxygen requirement of >30% and/or positive pressure (breathing, nasal CPAP or HFNC) | Oxygen requirement of >30% and/or positive pressure (breathing, nasal CPAP or HFNC) |
But what if you get complaints at a later age?
These criteria no longer apply to adults. And there are currently no guidelines on criteria for adults. There's an adult check to see if there's any lung damage. How long a baby has been ventilated is unrelated, as is the duration of pregnancy. It happens that adults born 26 weeks have no lung damage, but someone born 34 weeks has a lot of damage. This is because a foetus is born with 5% of the alveoli up to 36 weeks of pregnancy. The remaining 95% will be developed over the next 18 years. Early birth affects this, as does birth weight. Babies born too light (<2kg) may also have lung damage.
Adults may not officially meet the criteria of diagnosis BPD, but they may have lung damage due to premature birth. They then have poor lung function and abnormalities on a CT scan.
The CPL policy diagnoses lung damage caused by premature birth on the basis of the criteria they establish:
- Is someone born premature (born for 36 weeks);
- Is someone dysmature (born weighing less than 2kg);
- The story of the complaints;
- There are abnormalities on the CT scan and/ or lung function.
The policy nevertheless states that something must be measured/seen in, for example, the CT scan or lung function in order to diagnose it. In addition to early birth, the policy visit also looks further at possible other causes of the complaints.
Classic BPD vs. New BPD
Babies can be born ever earlier nowadays, already at 23 weeks. Doctors do see that the sooner someone is born, the more impatient the lungs are. This can also cause problems. Since 1991, babies are no longer intubated. This has caused the image of BPD to change according to science.7
Babies are now given a small breathing tube (MIST) in the trachea just after birth. Via a tube a substance is administered that helps to keep the alveoli open. This substance is normally produced from the 32nd week of pregnancy. The group that was still intubated often has improved lung development. However, this group often has more damage to the lungs itself due to the ventilation. Because of the high pressure of the ventilator, the alveoli are blown to pieces. In the group without intubation there is often unripe lungs. So there is less damage, but lungs are less mature.
In short, the classic BPD involves lung damage and fibrosis due to mechanical ventilation. While in the new form of BPD there is a disorder in lung development, because the lungs are less mature. 8 The doctors currently see no difference between the two groups named above. There is no difference in the image and no difference in the complaints people report. Also, there is no difference when doing a function test for the lungs. The doctors do expect that there is probably a possible difference in the lungs themselves. It's just hard to investigate this for now.
What tests are being conducted to determine lung damage by premature birth?
In order to determine lung damage, a number of studies are carried out:
CT scan
a lung function test
Depending on the complaints someone experiences, other investigations may be considered:
Blood tests on indication
An exercise study on indication
Sometimes a sleep study can be performed or an examination with the ENT doctor as sleep apnea is more common. There may even be references to other doctors. The heart is also often examined by the cardiologist, as shortness of breath can sometimes come from there. Furthermore, there is a higher risk of pulmonary hypertension, i.e. high blood pressure in the pulmonary vessels. This is more common in young people than in the elderly, but it is not known why. Each patient is different, and so, depending on the story someone tells, certain studies are done or not. The pulmonary doctors indicate that it remains important to take the complaints seriously and to give the person with the complaints recognition and recognition for his or her story.
What treatment is possible in case of lung damage due to premature birth?
In this form of lung damage it is important to know that it is often relatively young adults who are diagnosed. Often this group wants to be able to do as much as possible and to have a good quality of life.
However, it is not yet known what the exact course of this lung damage is. In a normal situation, the function of the lungs decreases slightly as people age. In the case of lung damage due to premature birth, it is not yet known whether this happens in the same way, for example, or can deteriorate faster.
The doctors will therefore indicate:
What is good must remain good as long as possible. .
In other words, it is important to keep lung function at the same level as long as possible so that people can continue to function as long as possible.
The following points are important:
- Lifestyle
- Healthy Weight
- Do not smoke or vap
- Medication
- Move enough
Lifestyle:
For people with lung damage it is important to pay attention to the lifestyle. Among other things, it looks at how someone eats, moves and smokes.
Especially moving can be a challenge. Especially in case of lung disease it is important to move to maintain the condition and strength. People often experience being short of breath during exercise or exercise, sometimes preventing them from creating the right exercise incentive. Some things are important when moving: breathing, condition and strength.
Breathing:
People with lung damage may have to do with rapid breathing and are often short of breath during exercise. It also often takes more energy to breathe because the lungs are sometimes less developed or have suffered damage at an earlier stage. There may have been damage to recurrent respiratory tract infections causing scar tissue and adversely affecting lung function. This can lead to high breathing, with high breathing you breathe more air than out, which can lead to bloating and raised shoulders. This makes inhaling harder and can make you feel stuffy. Also chest pain can be caused by stretching muscles and tendons. It is important to make people more aware that they can breathe from their belly, so that the diaphragm is more activated. A greater focus on exhalation can help in this. It can also help to train the internal respiratory muscles using, for example, IMT (Inspiratory Muscle Training). However, little literature is known about lung damage due to early birth and IMT training.
Sometimes there is air-trapping, or places in the lungs where air is left behind. This ensures that the lungs cannot ventilate properly and that people do not exhale properly. As a result, people have the idea that they need to breathe more and therefore become even short. A simple exercise like blowing with a straw in a glass of water can already help to stimulate the ventilation of the lungs. This causes pressure in the lungs and the alveoli. This pressure ensures that air is moved to the place where the least resistance is, and thus the ventilation of the lungs can be stimulated.
Condition:
In order to keep up the exercise there must be a good condition. When people start to move less because of lung disease, the condition deteriorates. The condition can be trained in different ways and also in different forms. An important point for building condition in lung disease is the way of training. This is best done through interval training. In other words a short peak of 1-2 minutes with intensive movement and then 2-4 minutes at a quiet and normal pace. This way the lungs have the chance to recover from the peak and it is easier to control the breathing. So these rest and recovery moments are very useful to the lungs. Especially if there are diffusion disorders, interval training is more effective. A long-term workout that keeps you at a constant pace makes the lungs less likely to recover, which means that breathing is no longer controlled. The interval training is recommended for both young and elderly. Young people have the advantage of being more taxable and thus creating a higher peak moment. Older people benefit more from longer rest periods. An interval training can be applied in the daily walk. It is good to take between 5000 and 10,000 steps a day every day. In between you can briefly use a higher running rate to implement interval moments during a walk.
Performance:
It is important that there is sufficient muscle strength in the body. Especially the leg muscles are important to carry. The leg muscles generally require most of the body's oxygen. The moment the lungs get worse, the first muscle group gets worse faster. People often still do things with their hands and arms, but they lack the power with everyday things like getting up from the chair. Also, if the leg muscles remain strong, these reserves form for a later period. Strength is then best built in sets of 3-4x where you repeat an exercise 8 to 12x for the production of muscle mass. After that, a person may feel that it was heavy after an exercise. It is important to include muscle strength exercises in addition to general daily activities (for example, walking the dog or shopping).
Heart rate:
While moving, the heart rate rises. How far it rises depends on the person himself, the condition, but also on how intensive the movement is. During an interval training work is done with a peak moment. Within this peak, people can choose to work on the maximum heart rate. This is calculated by 220- age. It is possible to take a percentage of this and train around a particular heartbeat or in a particular zone.
Cough:
Coughing in case of lung damage may occur in combination with recurrent respiratory infections. People regularly suffer from (sticky) mucus, also called sputum. When this affects people, different techniques can be used to make coughing as easy as possible. For example, we can use crap techniques that can help to cough up the mucus easily. A flutter can also be used, this is a tool that makes the mucus looser via oscillations (vibrations). Furthermore, a PEP mask (a mask in which you breathe resistance) can be used to induce coughing. In case of a lot of cough it is wise to take this to a doctor for further examination or any medication.
If necessary, a (long) physiotherapist can help people to maintain and/or build up the strength and condition. The respiratory pattern or cough techniques within these treatments can also be considered.
Healthy weight:
People with lung disease can go both ways with regard to weight. They may become overweight or underweight. Overweight can be caused by less movement because they are short of breath. Underweight can be caused by people eating less because they are too short of breath. But moving can also cost too much energy to make people lose weight faster.
It is therefore important to keep a close eye on the weight and to stand on the scale with some regularity. If necessary, a dietician can also be enabled. They can help, among other things, with advice on healthy food and with arrival or loss of weight.
Do not smoke or vap:
People with lung disease always receive urgent advice to stop smoking/vaping. A number of lung diseases already involve lung damage. Smoking or vases damage these affected parts even more. As a result, someone with reduced lung function will develop even more symptoms where lung function will decrease even further.9
Smoking/vaping itself can already stimulate the lungs, making you cough more often and irritating the airways themselves. Smoking also eventually causes additional damage to the lungs or to COPD disease (in which the alveoli break) and lung cancer. The vaping is still quite new and where it was initially thought to be innocent, researchers and doctors now come back to that. Vapen causes inflammation in the lungs, which can cause shortness of breath and can actually cause damage to the lungs in the short term.10
In addition, recent research has shown that poisonous metals are released into the lungs as well as into other areas of the body. These heavy metals (lead, uranium and cadmium) can cause permanent damage in both the long and short term. It can lead to cognitive disturbances, behavioural disturbances, respiratory problems. Furthermore, lead mainly affects the heart, blood vessels and the brain. cadmium is even associated with different types of cancer.11
Stopping smoking/vaping can be difficult because of the addictive nicotine. However, every cigarette, cigar or vape you do not take is already profit for the lungs. This will reduce the rate of deterioration. The advice to stop is also given to healthy people and to people with lung diseases who have arisen in a different way.
In case of lung disease, it is therefore important to keep lung function as good as possible and not to cause further damage to the lungs by smoking/vaping.
Medication:
In case of lung damage due to premature birth, the medicine that works best has not yet been investigated. There is therefore no known data. Doctors who have experience in this form of lung damage prescribe medications that widen the airways. In cases where asthma is still involved, an airway remover with an anti-inflammatory can be prescribed.
Finally, it remains important that overall health does not deteriorate, causing more lung damage to people.
It is important that people consult a doctor more quickly in case of infections. In this way, targeted action can be taken more quickly to prevent additional damage to the lungs.
It remains important to learn from each other, because the doctors themselves also learn from their patients. The doctors examine the complaint and can help others using experience stories from the patients. An example of this is someone who wants to walk in the mountains. At sea level (the level we live on in the Netherlands) we have an oxygen percentage of 21% in the air. When you go into the mountains, that level is lower, so the air is thinr, so you take less oxygen. A lung function test may indicate that it will be less good from a certain height. But people with (a suspicion of) lung damage due to early birth can already indicate at a lower height that for example breathing is not going well anymore, while according to the test it should go well. This has partly to do with the fact that people with a certain lung function have a low oxygen level in the blood faster, which makes someone gets complaints faster. It is not entirely unlikely that there is anything else going on that is not yet measurable, but what is there. This is also unknown territory for the doctors.
What is the course of lung damage due to premature birth?
How the image of lung damage will develop over the years is not yet known. It is also not yet known whether it has certain consequences at a later age.
However, the lung doctors know that women with this type of lung damage are more at risk of problems during pregnancy. In addition, there is a greater risk of premature birth.
The doctors indicate that it is good to work together with the gynecologist as a pulmonary policy and to think about issues such as complications and safety for mother and child during pregnancy.
It may be that a pregnancy isn't going well due to lung damage with the mother. Sometimes it is even necessary for the baby to receive extra oxygen during pregnancy and sometimes it can be associated with absorption. The human body regulates that the mother's body first gets enough oxygen and only subsequently to the baby. The extra oxygen can help to make sure the baby gets enough oxygen.
The pregnant woman is often more susceptible to infections, for example. Also, the diaphragm is higher during pregnancy, which can cause extra shortness of breath (light shortness of breath occurs with almost every pregnancy). Furthermore, pregnant people suffer more quickly from symptoms such as: stomach acid, cough and respiratory infections.
Sometimes heart problems can occur due to increased heart load due to pregnancy. The delivery itself is a greater risk. It is often best to give birth naturally. In a C-section a large wound is made. This can make breathing more difficult. Also the breathing is less good, coughing up often does less well and there is a greater chance of infections. There's a greater vulnerability to infections, especially in bad lungs. It is therefore preferable to follow the pregnant woman with lung damage through the hospital and not through the midwife or the general practitioner.
This page was created thanks to information provided by: Dr. Lieke Kamphuis (longarts), Drs. Arnold Duinisveld (longarts), Drs. Lidewij Visser (longarts) and Steven Huizer (long physiotherapist and geriatriefysiotherapist).
*A modified version of this original text has also been published on the Care4Neo website.
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- Erasmus MC. (2023). Bronchopulmonal dysplasia in adults. Collected from Erasmus MC: https://www.erasmusmc.en/en/patient care/diseases/bronchopulmonal dysplasia-adults#272ad53d-e1e7-4647-9b53-5d77acd503eb)
- Vrijlandt, E., Gerritsen, J., & Duiverman, E. (2007, November 3). Broncho-pulmonal dysplasia in ex-premature adults. Dutch magazine for medicine.
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- Dutch Association for Paediatrics. (2020, December). BPD. Picked up from Dutch Association for Paediatrics: https://www.nvk.nl/themes/quality/directives/directives?componentid=120029184&tagtitles=Cardiology%2Cintensive%252bCare%2Clong Diseases%2CNeonatology
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- Bui, D. S., et al. (2022, February, 18). Association between very to moderate preterm births, lung function deficits, and COPD at age 53: analysis of a prospective cohort study. Lancet respiratory medicine: https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(21)00508-7/abstract
- Houterman, K., & De Goede, A. (2023, October, 3). Doctors raise alarm after large vape research: many young users. Fetched from RTL news: https://www.rtlnieuws.nl/news/nederland/article/5410827/trimbos figures-alarming-vapen-e-cigaret-vapes-young teenagers
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