The early years
Once home with your baby you can start to enjoy your new family addition and begin to get into your own routine. Some parents find that they are a little nervous about ‘going it alone’ after what may have been a lengthy time in hospital with Doctor’s & Nurses close by at all times and you may have worries and concerns to begin with. Depending on the services offered by the hospital, you may be visited by outreach nurses once you are at home, who will help you and offer advice and who you can contact if you have any concerns or questions. Alternatively, your Health Visitor should be able to help and advise you. Remember, never be afraid to ring your GP or the baby unit if there is anything at all that you are worried about. You may also find it helpful to chat to other families in a similar situation and we can help to organise this for you. Of course, you can contact us anytime too by contacting our freephone number 0800 731 6991.
Most babies recover from CDH very well and are left with very few, if any, lasting medical issues but this really depends on each individual case and severity. Babies with moderate to severe forms of CDH may have more challenging issues. How Healthcare professionals decide on the severity of CDH can vary and they may take different things into consideration.
As we begin to see more babies surviving CDH than when this condition was first recognised, we will probably discover and learn more about what types of issues or medical problems they may experience and hopefully how to resolve or treat them and as we now have more patients reaching adulthood we are able to witness and identify any problems they have developed or are experiencing later in life, so that we can help to improve outcomes for future generations.
Here are some of the more common medical issues associated with CDH, which with today’s medical advancements and treatments are better identified and managed by Medical Professionals, together with some other useful information on things you may have concerns about.
Reflux is caused by the valve which opens and closes when we eat not doing it’s job properly and allowing food, drink and acid to rise from the stomach into the food pipe and sometimes up into throat, resulting in discomfort and sometimes regurgitation of stomach contents or vomiting. This is usually mild in most cases and your baby may display some discomfort and create more washing for you! but it is normally not a major issue and can be treated effectively with medication prescribed by your Consultant or GP. Sometimes the Reflux can be severe, causing baby and parents a great deal of distress and can result in baby not keeping food & drink down long enough to benefit nutritionally. There is also the risk of milk or food aspiration, whereby when the milk or food rises up the food pipe and there is inhaled into the lungs, which can be serious. In these cases your baby or child will be referred to a specialist who may recommend surgical treatment to resolve the reflux. The procedure to resolve reflux is called a Nissen Fundoplication. This procedure enables the surgeon to improve the natural barrier between the stomach and the food pipe (oesophagus) by wrapping a part of the stomach known as the gastric fundus around the lower part of the food pipe. This prevents the flow of acid and or food etc. from the stomach into the food pipe, and strengthens the valve between the food pipe and stomach, which stops acid from backing up as easily. This procedure is often done using a surgical instrument with a camera called a laparoscope. This is like a thin telescope with a light source and is used to light up and magnify everything inside the food pipe and stomach so the surgeon can see things clearly. A fundoplication can also be done as traditional (open) surgery.
Some children who have been born with CDH sometimes have difficulty feeding. This can be for a variety of reasons from the initial long NICU stay, accepting volume of feed, reflux, not liking things in or near the mouth (sensory issues) or a combination. These children will sometimes need some extra help. This extra help may be from a Dietitian or Speech and Language Therapist (sometimes called SALT), or both. The extra help may sometimes be by a feeding tube. There are different types of tube, such as an NG tube (Naso-Gastric), which passes through the nose and into the stomach, or a G-tube (Gastric), which is inserted through the skin on the tummy and directly into the stomach. These are the most common types of feeding tube. If reflux is diagnosed then medication may also be required and sometimes a special thicker milk or high calorie milk, which is usually prescribed. This is often sufficient to manage the condition, however occasionally the reflux may be severe and a Nissen Fundoplication is required to assist your child in keeping the food down. Some children find accepting the required volume of feed difficult and sometimes a feeding pump that drip feeds food may be introduced for a period of time while your child grows and can accept more food. All these measures can help if your child is not putting on weight and height as they should. Sometimes this is called a ‘failure to thrive’. You may also be referred to a Dietician who will be able to advise you of ways to help improve weight and growth.
Feeding issues can be very distressing for both you and your baby and make you feel alone. Here at CDH UK we have lots of people who have experience of this and ‘we are always here’ should you need to talk. We also have additional information leaflets on feeding difficulties.
Some Children who have been born with CDH sometimes have a slightly increased risk of developing Asthma. It is extremely important that if your child has any difficulties breathing that you take them straight to your Doctor for diagnosis. There are now many medications that can be used to help control asthma and if used correctly can be of great benefit to your child. Some parents report that their CDH child appears to breathe much faster than normal and this should be discussed with your paediatrician. Most often it is simply because of their lungs being smaller and nothing to worry about as it improves as their lungs grow. CDH children appear to have a higher incidence of chest infections than other young children, so it is important that you consider having them vaccinated against flu. CDH children who come home still on oxygen are particularly at risk of all the winter bugs and you should seek advice from your medical provider on what vaccinations may be beneficial.
You may notice that your child suffers more than other children from chest infections or they may experience recurrent chest infections. These will be treated accordingly by your GP or Consultant and you will usually find that things improve as your child grows up. Don’t feel afraid to take your child to your GP if you feel that they may have or be developing a chest infection even if it turns out to be nothing. It is always better to go with your instinct and be cautious. Sometimes your GP or Consultant may prescribe a small continuous course of antibiotics as a preventative measure and this is known as prophylactic antibiotics.
It has been reported that some CDH children have an increased risk of developing Scoliosis (curvature of the spine) It is therefore recommended that this is monitored by your Consultant or GP to detect it early, so that it can be managed should it progress. Mild forms of Scoliosis are managed conservatively which means that they watched carefully to avoid surgery. Some cases may require physiotherapy or a rigid vest or cast that is worn for a set period of time to guide the spine or prevent the curve from getting worse. If surgery is required in a severe case then this normally involves inserting metal rods to straighten the spine and maintain it.
Occasionally babies born with CDH can develop hearing loss. Nobody is really sure why this occurs, but it is thought to be linked to artificial ventilation. Your child’s hearing will be monitored on a regular basis to ensure that no hearing loss has developed. Sometimes CDH children can also suffer speech delay and may require speech therapy.
Some children show some signs of development delay. This is sometimes noticed more in children whose hospital stays have been prolonged. Nobody is quite sure why this occurs, but it is thought to be again associated with artificial ventilation and delayed maternal contact. The type of delays can be varied from sitting up late, a delay in walking, to speech and language delays and sometimes learning delays when they are older. If you have any concerns about your child’s development please make your paediatrician or GP or Health Visitor aware of your concerns. If they feel it is necessary they will refer you to a specialist who deals with Child Development.
Occasionally some CDH children will re-herniate. It is important to recognise the signs should this occur –
- Sudden onset of breathing difficulties
- Severe vomiting; especially green bile, indicating there may be a twist in the bowel which may need surgery
- Any abdominal discomfort
- Generally feeling lethargic & unwell
Please seek medical advice immediately should you suspect that your child has re-herniated . If your child has re-herniated then a second repair normally takes place to repair the diaphragm. This is normally fairly straight forward and your medical provider will be able to discuss all the options with you and schedule a time and hospital for this to take place.
Every year, especially during the Winter months we see the arrival of different types of viruses such as; Norovirus (sickness & Diarrhoea) the common cold and various Flu viruses. These viruses can be extremely worrying for parents especially considering a lot of CDH children already have feeding issues and breathing problems, which can be made much worse by these viruses. Parents can become overly stressed and this can lead to not wanting your child to be in an environment where there are lots of people, such as parties, family gatherings and even school. This is understandable, but there are steps that you can take to reduce the possibility of your child contracting one of these viruses (although no guarantees!). Obviously strict hygiene standards, such as not sharing cups and cutlery, food or drink and ensuring that hands are thoroughly washed before and after meals and after visiting places and of course after nappy changes and visits to the toilet. Ensure that runny noses are kept clean and that coughs and sneezes are caught in a clean tissue, discarded in a bin after use and hands are washed. You may also discuss available flu vaccines with your GP. It is also advisable to asks friends and family not to visit if they have viruses or have been in contact with anyone who has displayed symptoms of a virus and to avoid areas or places that you know have had incidences of viruses such as hospitals, playgroups etc. if at all possible. If your child does become ill from a virus seek the advice of your GP by phone (you don’t want to spread it by visiting the surgery, if this can be avoided) and if symptoms persist or become worse request a GP visit or call NHS Direct 0845 4647.
Some CDH babies do not tolerate lumpy weaning food well, and may progress straight onto finger food from puree. Some tend to have an overdeveloped gag reflex and can be sick very easily. This can be quite alarming at first, and it may be a good idea to tell school or nursery this.
Lots of CDH toddlers do not eat huge quantities and prefer to graze, and they may well develop food aversions, perhaps not eating hot food or certain other foods at all, and sticking to a small range of foods; yoghurt, fruit, cucumber etc. It can be frustrating to cook family meals and your child eat nothing. Try not to worry, children will not generally starve themselves!! Try not to think that your child is being deliberately awkward, or at least give them the benefit of the doubt. Just try to let your child eat what you know they like, knowing that this will mean often making them separate snacks at family meal times whilst at the same time giving them the opportunity to eat what everyone else is having. If your child has been tube fed for a long period of time, especially if via NG tube, they may associate food with their noses, and therefore may try to push food up their nose instead of putting in their mouth! Be vigilant of this when weaning them onto finger food, and especially if your child likes small items of food such as raisins, smarties or other small items.
CDH children at school
Make sure your child’s school have all the information they need regarding your child and CDH. Print it off and send it in every year, and make sure all staff in school are aware exactly what CDH is and what your child’s individual situation is. Include emergency contact numbers. Check any medication in school is in date regularly and replace if necessary. It would be wise to get an annual flu jab. Most CDH children can do PE, go swimming, and join in all normal school activities. If they have school, dinners, perhaps the lunchtime staff will keep a “dinner diary” telling you what your child has eaten and how much they have managed to eat. If your child has more health issue’s then you may require them to have a medical statement or if there are development delays then an SEN (statement of educational needs). Your local school and LEA will be able to help you decide if you need to to this.
Out and about
There is no reason why CDH children cannot go on holiday, fly long/short haul, go camping, swimming etc. It is up to you to decide what your child can cope with (and you!). You must declare CDH as a medical condition for insurance purposes, and may have to pay an extra premium for travel and holiday insurance. Make sure your insurer knows exactly what CDH is as some don’t. Generally your GP will give your Child a check over before you travel if that will put your mind at rest.
Unfortunately some babies and children do have more complex and lasting medical problems such as Heart Defects, Oxygen dependency, brain damage, bowel/intestinal damage, total hearing loss, or even a failure to thrive, to name a few, but thankfully these are not common.
To give an insight into adulthood we have included the following two extracts from stories written by adult CDH survivors.Their experiences are individual to them and every CDH survivor is different and cannot be compared.
Looking at my teenage years growing up I think myself quite lucky really, I did find sport quite difficult as when I exert myself I found it hard to catch my breath, and had to sit out quite a lot, also I used to get a ‘stitch’ in my scar, which was extremely painful, so mainly I was only able to take part in the more gentle sports! I found sports day quite difficult, which used to single me out! I did get bullied quite a lot during my school years due to my scaring, I was called ‘the patchwork’ girl or they used to say that my parents had tried cutting me up! and also because I was always very small for my age, that didn’t help either! Kids eh!.
As I got older that obviously stopped, but I was very self conscious about my body! When I started getting boyfriends that was very difficult too, but as I was older I was a bit more forward about it, I used to say ‘here look at my scar’ I was still very conscious about it though. Medically I think myself very lucky really compared to a lot of CDH survivors, I had to take medication for reflux every day, and also Gaviscon. But as a rule I’ve led a pretty normal life!
When I met my husband, we went out on a few dates and got on very well, he has scaring on his left hand due to a burn injury as a baby, I asked him about it, and he said he also had a lot of scaring to on his leg also. This is in a funny sort of way made me feel a lot more comfortable about my scaring! (something else we had in common)!! I got pregnant at 21 with my daughter Rhea, it was quite a shock, as I was always led to believe that I may be unable to have children, but she was a lovely surprise! In early pregnancy I saw a specialist consultant, I had my first scan at 9 weeks and from 16 weeks I had scans every 2 weeks, one to make sure everything was OK, but also to see if the baby had CDH too.
As I was starting to get my bump, where I was growing my scar was extremely painful, my main scar is stuck to my ribs. I saw my consultant again, and he was telling me that if ever I wanted anymore children, he could ‘cut out my scar’ and give me a new one! Back then I wasn’t planning anymore! He also said that I wouldn’t be able to go full term as I couldn’t grow to full term! In pregnancy I developed diabetes which also made the pregnancy harder! By about 20 weeks I was in a lot of pain with my scar, but I wasn’t happy about taking drugs, I used a TENS machine and had an awful lot of bed rest. By this time I was on a bottle of Gaviscon a week, reflux was a huge problem as it is in a ‘normal’ pregnancy. But it was very extreme!
They decided to induce me at 36 weeks, an was told possibly I’d have to have a C-section as me pushing would put a lot of strain on my already stretched scar.
I went into the hospital to be induced at 36 weeks! I couldn’t move around very much! I took them all by surprise and gave birth to my daughter at 6.40am! I was delighted to have been able to give birth naturally! Amazing! My daughter was born perfect, no problems, no CDH. All she had was a feeding tube for 36 hours as she didn’t have the sucking reflex due to being premature!
I think myself so very lucky and proud to have been able to give birth to my daughter, with limited problems!
3 years later I had my son, I didn’t choose the option of having my scar cut out, as I thought as it had been stretched once already, it would be fine again! This pregnancy was almost the same as my first until about 20 weeks and I developed pubis synopsis. This was added pain I really found difficult, so yet more bed rest and was on crutches! And yes the diabetes came back! Grrr (which has never gone since having him).
I had all the usual 2 weekly scans as I did with Rhea. The consultant again said I couldn’t go full term. And he strongly recommended a C-section at 36 weeks! I went into labour at 34 weeks in the middle of the night, and he was born at 5.20am! And too late for a C-section! I was shocked really as it all happened so quickly! But as he was premature, born at 34 weeks he was taken straight to SCBU, he was on a ventilator and was gravely poorly, but 4 weeks later he came home! No CDH! He did have breathing problems though, and was a very poorly baby. At 6 weeks (on boxing day, his due day) he stopped breathing, I had to administer CPR which was the worst day of my life, he was airlifted to hospital and after 3 weeks in HDU he came home again.
Ayrton is now 7 and very healthy and Rhea is now 10 and also healthy!! After 2 healthy babies, no more for me! I think myself so very lucky to have had them and got through 2 pregnancies. And all I’m left with is the diabetes! A small price to pay! I still have to take reflux medication every day.
I have now in the last 18 months been suffering with Coxyodinia, really really painful. I saw my consultant just before Christmas and am waiting to have an epidural put into my coccyx. In the beginning they put it down to childbirth, but when I saw her in December she seems to think its something to do with all the problems as a baby. I’m not sure, and to be honest I’m not worried about what has actually caused it.
Dave was born in September 1962 with no pre-natal diagnosis of CDH. He began projectile vomiting aged 3 months, and wasn’t putting any weight on at all. He was taken to hospital and his Mum kept pressuring them that there was something wrong, and finally he was diagnosed with CDH.
His CDH was operated on and eventually, Dave pulled through. It took quite a long time for Dave to get fit and well, and he had lots of check ups.
Dave has been fine all these years, and tells everyone his scar is a shark bite from Australia.
This is what Dave’s Mum has told him about his start in life and about his CDH.
Dave is 48 now, and hopes that his story will bring hope to parents and families of CDH UK toddlers, teenagers and beyond.
So as you can see from these extracts life for a survivor of CDH can vary greatly and as more and more people survive this devastating defect the more evidence will be compiled as to what to expect in the future.