Respiratory

16/3/05

Respiratory Problems 

Children with EvC have lungs which are smaller than normal because they’ve had to develop within a small ribcage. This can be very mild so your child has no problems at all, however some are really dogged by problems because their ribs and lungs are so small.

• Ribcage CT scan - front view rotating

• Ribcage CT scan - sideview rotating

• Lungs - multiplanar rotating. Please forgive the black areas. My software is limited for editing this.

• Lungs - supine view rotating. Ditto regarding the editing.

EvC also affects the way the airways develop so the walls may be softer than normal – tracheomalacia or bronchomalacia (same as tracheomalacia, but involving airways further down the lungs) - unless severe, your child won't have problems with this. 

Your baby may breathe more quickly than normal – tachypnoea. You may notice that his/her ribs get pulled in at the lower edge – subcostal recession. As long as s/he is nice and pink this shouldn’t be cause for concern.

If the lungs and ribcage are very small at birth your baby may develop pulmonary hypertension. If very severe, your baby may need to be ventilated and placed on nitric oxide, a gas which dilates the blood vessels around the lungs, thus reducing the pulmonary blood pressure. If less severe, then medication and monitoring may be all that’s needed. As your baby gets bigger, these problems usually improve, but there are no guarantees unfortunately.

Quite a few children with EvC are prone to chest infections and asthma for the first few years. This does improve in time, but it varies from child to child so I can't give you an age here.

Gastric reflux is an issue that can affect your child's lungs and airways - the stomach acid coming up the gullet can go down again the wrong way and cause chronic inflammation of the throat and trachea. If you notice that your baby or young child coughs and gags a lot after meals or when lying down flat, please bring it to the attention of your doctor. There are several treatments available for reflux. 

More Severe Respiratory Problems and Solutions

This section is really only for those of you whose children are going to have long term issues with respiratory problems. If you've just had your baby and they're at home with you and doing fine, please don't read this and fret that it will happen to them. You will know if your child is going to have serious issues within the first 3 months.

Tracheostomy

This is a measure used for children whose airways are very floppy and will not stay open, for children who have scar tissue blocking their airway caused by the trauma of intubation at birth (usually premature), or for children who require long term ventilation. It involves creating a hole (stoma) at the front of the throat, then placing a tube within that stoma to keep it open. The tube is held in place with tapes which go round the child's neck. The operation is very straightforward and complications are rare. Your reaction to your child needing one will depend on many things. Some people are really traumatised by it, others are just really relieved that something can be done to improve their child's breathing. I was in the latter category since Angus had been in hospital for 3 months already with ventilation or CPAP through his nose. This really restricted his movements, and we couldn't see his face properly. I felt a great sense of peace seeing him after his surgery. He was more stable after it so the medical staff reduced the level of ventilation and oxygen required. If your child has been struggling to breathe with retractions of the chest, stridor or blue episodes, you will see a different and happier child emerge after trach surgery.

What happens next? Well, in the weeks immediately after trach surgery you will wonder how you are ever going to manage because kids usually need a lot of suction to keep the tube clear of phlegm/lung secretions. They produce a lot of secretions initially because their trachea is not used to having a tube in it all the time. It does settle down in time. Suction is a simple procedure in principle until you come to do it the first few times! I think all parents feel pretty hamfisted then, but it does become a lot easier especially since you'll get so much practice at the beginning. The medical staff will teach you how to do suction, what signs to look for that your child needs it, how to take care of the stoma and keep it clean. Don't worry if there are times you feel like freaking out or do actually freak out - this is completely normal and lots of parents of kids with trachs have done just the same. The medical staff know this and will not judge you harshly for it. Be kind to yourself, regroup, and try again.

Tube changes are a part of trach life and probably one of the most nerve racking to start with. How often they need to be done depends on the type of tube and your child. When Angus first got his trach he needed it changed every 1-2 days because it kept getting blocked. We weren't allowed to do his tube changes until his stitches came out. I was pretty nervous about doing my first tube change because I didn't know what his stoma would look like, and what his reaction would be. Would the stoma look really gross? Would he cough and constrict his stoma so I couldn't get the tube in?? It went without a hitch fortunately. His tube changes have always been very simple since.

By far the best resource on the internet if you have a child with a trach is Aaron's Tracheostomy Page written by Cynthia Bissell. She is a nurse who had a trach herself as a child and had a son who needed a trach. Everything you could possibly want to know about your child and trachs can be found there. There is also an excellent support forum where you can compare notes with other parents.

Ventilation

If your child has severe heart and/or lung problems then they may need the help of a ventilator. I was utterly miserable and overwhelmed when I was told that Angus needed long term ventilation for "an indefinite period". I could not picture how he or our family could have any kind of life with him attached to a ventilator and oxygen. If you feel like your life is over when you get that news, please do not despair or be hard on yourself for thinking that way. It took me weeks to get to grips with that bombshell. What I needed to realise at the time is that our adjustment would be gradual. No-one was going to toss him out of the hospital with all that medical equipment and leave us to get on with it! No. We got lots of training, he had a care team assigned to him (that took a very long time), and he came out of the hospital for an afternoon here and there, then every weekend, then to nursery in the mornings and home every weekend, then finally home for good. This enabled us to get to grips with what we needed to take with us whenever Angus was with us - a "To go" bag with all his suction gear, spare tubes and emergency equipment was a must, oxygen cylinder plus spare, spare ventilator, spare batteries, feeding kit etc. It was really daunting, but it slowly became second nature. At home we made adaptations like a little trolley that the ventilator sits on (thanks dad!). It was made from wood we had hanging about in the garage, 4 casters from a hardware shop and a piece of non-slip matting.

He can wheel this around independently, though he struggles on carpet. He goes to nursery 5 days a week and loves it. He can walk, talk, be a "normal" little boy within the confines of his ventilator hose. We go shopping, to restaurants, softplay areas, and on family outings. I can take him trips all by myself. It's not nearly as limiting as I thought at first. It can be hard work, like the time we carried him in his buggy with vent and oxygen over a marsh, then I slowly pushed him up a grassy hill so we could fly kites at the top. It was worth all the puffing and blowing (on my part!) to see his face when we got to the top and unfurled the kites.

Surgery

If your child has serious cardiac issues then cardiac surgery may solve/improve their breathing problems. The way the heart and lungs work together means that one not working well, affects the other. If their cardiac issues are mostly sorted and they are still not making significant progress off the ventilator by the age of 18 months, start pushing for surgical intervention to expand their ribcage. There are two main options for this -

• VEPTR (vertical expandable prosthetic titanium rib)

• LTE (lateral thoracic expansion)

VEPTR

This is a modified version of the VEPTR done for correcting scoliosis (side curvature of the spine). In the version done to expand the ribcage, the ribs are cut, and a C-shaped, vertical, expandable, titanium strut is hooked over the top and bottom rib at the back. The surgery is done one side, and 6 months later the other side. Thereafter the struts are expanded slightly 2-3 times a year - this is a surgical procedure and requires general anaesthesia. You can find out more about it here including surgeons in your area who are familiar with the technique.

LTE

This is less commonly carried out since it is only helpful for children with narrow chests, e.g. EvC, Jeune's, Barnes. It is associated with fewer post-op complications than the VEPTR and does not require repeat surgery to adjust devices, though it can be repeated if the child needs further expansion when they get older. I chose this option for Angus because I did not want him to have repeat surgeries, I liked the fact that there was less to go wrong post-op, and it also seemed a more logical option mechanically. You can find out about the operation here. This operation is available in -

• Columbus Children's Hospital, Ohio, USA

• A hospital in Hollywood, Florida (I will find out details), USA

• Royal Alexandra Hospital, Westmead, Sydney, Australia

• Soon to be available in Great Ormond Street Hospital, London, UK