The desire to have children can be incredibly strong. It can be something that we’ve dreamed of for a long time, or it can be something that creeps up on us as we get older. Sometimes life events start us thinking about children, such as the death of someone we know, or finding a ‘forever partner’. The urge to reproduce is something that is in our most basic instincts so it’s understandable that most of us, regardless of health, wealth or cultural background, reach a point where we think about whether we want to build a family around us.
For some women with PH, that decision is very easy and straightforward. They do not want to risk a pregnancy and they are content with the friends and family structure they have around them. For others, who again are not happy with the high risk of pregnancy, there are increasing options for having a family that include adoption, fostering or surrogacy. And finally, we know there are a proportion of women with PH, who are aware of all the risks, but still wish to try for a successful pregnancy.
What you need to consider
If you are considering getting pregnant, there are several things to think through.
Statistics are tricky things- they can be interpreted in differing ways and sometimes lull us into a false sense of security. A condition that occurs one in 100,000 people doesn’t sound very serious, but if you are that one person, it can be very serious indeed. Because pulmonary hypertension is rare, there are relatively few numbers of women who have been through pregnancies. This means the statistics that are available are based on very small numbers and are therefore harder to interpret.
The most significant risk for a woman who is pregnant and has PH, is the possibility of the loss of life of the woman.
If a woman’s pulmonary hypertension is poorly controlled before and during pregnancy, or if the woman has severe pulmonary hypertension, the risk will be significantly higher. Even patients who are very well can deteriorate during or shortly following delivery and die. Currently, there is not enough information available to identify which women would have better outcomes and therefore a low risk.
A one in five chance may seem a risk you can accept, after all, that means four out of five women will survive. But it really is worth thinking through the reality of what life would look like for those closest to you if you were one of the 20%.
How would your partner, or children you may already have, or parents, or friends deal with the loss? And do you really feel ready to let go of life yourself? Are there other dreams or ambitions you have yet to realise?
Dealing with problems during pregnancy
Another difficult area to think about is how you would deal with problems during the pregnancy. Your PH symptoms may be well controlled before you become pregnant, but there is a real possibility that they may significantly worsen as the pregnancy progresses.
If your symptoms become life threatening, your team will offer you a termination of pregnancy. If you deteriorate early during pregnancy, before 24 weeks, then without termination there is very high risk of the woman dying.
Some patients may start deteriorating for the first time around the 20-24 week time. At this time, the termination would be a medical termination and involve being induced and delivering the foetus. It’s really important to think about how you would feel in this situation, and if you could make – and live with – the hard decision to terminate the pregnancy in order to save your life.
Remember the medical team will always put your health over that of an unborn baby. This is something that is standardised throughout the world and therefore no matter what your wishes are, even if you would want the team to prioritise your unborn babies needs over your own, they will always be obliged to put your health needs first.
Having thought through all the different scenarios and challenges that can be faced, planning a pregnancy may still seem like an option you want to try. If this is the case, do not be embarrassed or ashamed to bring this up with your team, just be honest.
The importance of planning and communication
A planned pregnancy is more likely to give better results for mum and baby than an unplanned pregnancy – regardless of whether you have pulmonary hypertension or not. So, just as all would-be-mums are encouraged to think about their health pre-pregnancy, as a woman with PH you should be involving your medical team before trying to conceive. This is to ensure you are as fit as you can be and your PH symptoms as well controlled as they can be.
Your medical team will want to be sure that you understand the risks of a pregnancy. They may want to introduce to other members of the team such as obstetricians and anaesthetists who will be closely involved in your care during any pregnancy, and they will want to review your symptoms and your medications.
As previously mentioned, some medications are not advised during pregnancy, so if you are on one of these, talking with your team early enough gives you time to smoothly change medication and stabilise your PH again. If you make decisions yourself and stop medication without telling your team, you run the risk of destabilising your pulmonary hypertension symptoms and thereby reducing the risk of a successful pregnancy.
What care to expect during a pregnancy
Any pregnancy for a woman with PH will be classed as “high risk”. This means that there will be more frequent appointments throughout the pregnancy and that the care will be led by doctors (obstetricians and PH consultants) rather than midwives. It will also mean frequent appointments as both the pregnancy and the pulmonary hypertension will need to be monitored.
A common pattern of care would be appointments every four weeks from the start of the pregnancy up to 28 weeks’ gestation. From a PH perspective, the team will be wanting to monitor your PH symptoms and check how well your heart and lungs are functioning by examining you, checking an ECG, carrying out exercise testing and at selected visits arranging an echocardiogram or MRI scan.
From a pregnancy perspective, the team will want to see how you are managing with any pregnancy related issues and will want to ensure the baby is growing well with regular growth scans.
After 28 weeks, appointments will likely be every two weeks. Again, the PH team will want to be ensuring your heart and lungs are functioning as well as they can, but also attention from the obstetric team as well as the PH team will start to turn to the delivery of your baby.
In PH, delivery of a baby is normally planned for about 34 weeks. This may seem very early, but generally babies born at this gestation do very well and have very few long-term issues related to their prematurity. The delivery itself will need a large team of different professionals; from PH physicians and nurses, to obstetricians and midwives, to paediatricians and to anaesthetists.
Because of this, a spontaneous labour which could happen a long way away from hospital or when it’s not possible to get hold of the large team needed, is something to be avoided. This is why the baby is delivered six weeks early.
The safest way to deliver the baby is by an elective Caesarean section. Vaginal delivery may be considered in women who have had children previously and have a history of straightforward vaginal deliveries, but the main problem with a vaginal delivery is that it increases the cardiac output. As mentioned in an earlier section, the cardiac output is the amount of blood that the heart pumps through the body and for people with PH any increase in cardiac output can be challenging as the heart is already working under strain. With all the other changes happening during labour and delivery, the strain a vaginal delivery could put on a heart of a woman with PH makes it more dangerous than a Caesarean section.
You will normally be admitted to hospital one or two days before the planned delivery. This gives the medical team a chance to prepare things as much as they are able to. You will be given an injection of steroids which helps prepare the baby’s lungs for birth.
On the day of delivery you will be prepared for theatre as a Caesarean section is an operation which is carried out in a full operating theatre. You’ll need to be very closely monitored during the operation so the team will need to attach you up to different machines and monitors and you will need venous and arterial access. These provide ways into your blood vessels so that you can be given fluids or antibiotics or other medications as you need them and so that the team can take blood samples, monitor blood pressure and oxygen levels as well as other information. Some of these access points will be in your upper chest and others will be in your arms.
The type of anaesthesia normally used is an epidural which is classed as a regional anaesthetic and involves the anaesthetic being injected into your back. This numbs everything from your abdomen down both of your legs but means that if all goes according to plan, you will be awake throughout the delivery. If things don’t go smoothly, there may be a need to change to using a general anaesthetic.
Following the delivery, you will need to be on the Intensive Therapy Unit (ITU) for at least seven days. Although you are safely though the pregnancy and delivery, this is still a really risky time. There are lots of changes going on in your body and these can lead to significant strain on your heart and lungs. This is why you will still need lots of monitoring and medication during this time.
Assuming all is well and stable after a week, you will normally be discharged home at this time. But there will still be lots of appointments to attend for your PH over the next six months or more, as well as appointments that might be needed from the paediatric team for your baby.
Everything covered in this section is a general outline of the care you might expect during a pregnancy. Details are likely to differ between hospitals and inidividuals. Your team will put together a complete plan which is tailored to you and will cover all eventualities. You will have a copy of the personalised management plan to carry with your normal pregnancy notes, in case there are any issues when you are away from your normal place of care.
The plan will have specific ways of dealing with unexpected emergencies such as early onset of labour, emergency Caesarean sections and cardiac arrests. It will also contain detailed plans about the expected delivery- what anaesthetic drugs to use, who the team consists of, their contact details and the outline for the planned Caesarean section.
This may all sound complex and intense but having a baby when you have pulmonary hypertension is risky and complicated and will need very intensive medical care.