This information from Great Ormond Street Hospital (GOSH) is for parents of children and young people undergoing assessment for possible lung or heart-lung transplantation. A transplant is a serious operation and is not without risk. A transplant can be the only effective treatment option for certain serious lung diseases; however, it is not a cure. In many situations transplantation can lead to an extension of life with improved quality.Everyone will respond to a transplant in a different way. There are of course associated risks with transplantation, and while the early survival rates after the transplant are good, there can be no guarantees that any particular transplant will be a success.
Once your child has undergone their transplant they will have to take immunosuppressive (anti-rejection) medication every day, for the rest of their life. This medication prevents the body from rejecting your child's new lungs. Keeping your child and their new lungs healthy is a team effort. The team consists of you, your child, your family, nurses, doctors, psychologists and other members of the hospital staff.
Cardiothoracic Transplantation at GOSH
The Cardiothoracic Transplant Unit at GOSH has been established since 1988 when the first heart transplant at GOSH was performed. Currently the Cardiothoracic Transplant Unit at GOSH is the biggest paediatric transplant centre in Europe performing around 20 to 30 transplants per year. There is a full time team of 12 medical and nursing staff who will look after you and your child.
Types of Transplant
- Single Lung Transplant – In this operation a patient receives one lung (left or right), although this is rarely carried out in children.
- Double lung transplant (sometimes called bilateral lung transplant) – In this operation a patient receives two lungs. This is commonly performed for cystic fibrosis, bronchiectasis and pulmonary hypertension.
- Combined heart-lung transplant – In this operation a patient receives a heart and both lungs.
Survival after lung and heart-lung transplantation
During the early years of the lung and heart-lung transplant programme at GOSH (1998) approximately half of the children who received a transplant survived less than four years following transplantation.
In recent years, significant advances have been made in surgical technique, post-operative intensive care management, immunosuppressive therapy, and post-transplant surveillance. Since 2003, survival in children after lung or heart-lung transplant has significantly improved at GOSH, giving a survival of over 70 per cent at five years, and an estimated survival of more than 50 per cent by seven years. The risk for an individual patient may be more or less than the average depending on their medical condition.
The commonest causes of death during the first year after surgery are:
- Poor function of the transplanted lungs (primary graft dysfunction)
- Acute rejection
- Chronic rejection of the lungs
- Kidney impairment
- Malignancy (cancer)
- Coronary artery disease (in heart-lung transplant patients)
Lung transplant assessment
Your referring doctor thinks that your child may benefit from a transplant. We would like to see you and your child so that we can assess your child's suitability for a transplant. No child can be accepted onto the waiting list for a lung or heart-lung transplant without having been reviewed in person by members of the transplant team, which includes a paediatric transplant consultant.
Assessment for the transplant usually takes place over three to four days. Further tests or treatment may be required involving a longer stay. Your child may need to come back for the day as an outpatient, or be re-admitted for further tests. When you and your child arrive the first contact you will have is with the transplant specialist nurses. Your child will also be seen and examined by a doctor. During the next few days your child will undergo a variety of tests and investigations such as those listed overleaf. These are usually completed in three to four days.
The results are collated and your child's case will be discussed at the regular transplant assessment meeting also known as the Joint Cardiac Conference (JCC). This is a multi-disciplinary team meeting where the decision is made to place patients on the list or to order more tests where appropriate. In some cases to ensure your child is receiving maximum therapy, alternative treatment may be recommended. If you are not given a decision on your child's suitability to be placed on the waiting list before you go home, a member of the transplant team will contact you following your child's discharge.
During your child's stay, a member of the transplant team will ensure that you and your child have the opportunity to discuss the contents of this information. Not only does the information enable you and your child to make an informed decision regarding transplantation, but it is also a reference for you, your child and other family members as you go through the various stages of the transplant process. You and your child will be involved in all decision-making about going onto the Transplant List.
At the beginning of your child's assessment, we will not know for certain if your child will need a transplant, or if there are any reasons why transplantation would not be an option for your child. We will be able to provide you and your child with this information at the end of the assessment or soon afterwards. The transplant specialist nurse will explain the tests and provide support and guidance for you and your child throughout the assessment process. We will ensure that both you and your child have the opportunity to meet all of the relevant transplant team members, and are aware of the test results and what they mean.
Transplantation is a procedure with significant risks. Every child who is referred to us will be given the same thorough physical and psychosocial assessments to ensure that the procedure is the best option for them. Your child will be accepted on to the list only if the benefits of surgery outweigh the risks. If your child is not accepted then the medical staff will give a full explanation and advice on what to do next. In some cases an alternative treatment may be considered preferable, for example further drug therapy, medical management, other lung surgery or non-invasive ventilation.
If your child is accepted on to the transplant waiting list, they will be reviewed by the transplant team at a booster visit every six months unless your child becomes too sick to travel. If your child's condition deteriorates and their other body systems, such as kidney function, start to decline your child may need further interventions to optimise their general health before the transplant operation. If a change in your child's medical condition adversely affects the chance of transplantation being a success, your child may need to be taken off the list either temporarily or permanently.
The aims of the transplant team during this assessment admission include:
- Tests and investigations that are required to assess your child's suitability for transplantation
- When appropriate, alternatives to transplantation will be considered
- The opportunity for you and your child to ask questions and gain support
- The possibility for you and your child to meet other children and their families who have already had a transplant
- Ensuring that you and your child know the outcome of the tests and the decision regarding transplantation before discharge or when all test results are available
- That you know whom to contact if you or your child has problems or queries
Tests and investigations during the assessment period
Here is a list of the tests/investigations your child may have. Your child may have had many of these in the past. We will explain any unfamiliar ones to you and your child.
- Clinical observations, such as blood pressure
- Bacterial swabs
- Sputum and urine samples
- Chest x-ray
- Twelve lead electrocardiogram (ECG)
- Ultrasound (echo) of the heart
- Lung function tests
- Chest CT scan (which may require an intravenous cannula to be inserted)
- Blood tests
- Exercise tests
Additional tests may include:
- Cardiac catheterisation (angio - left and/or right heart catheterisation).
- Bone scan (DEXA)
- Impedance study
- Glomerular filtration rate (kidney function test)
- Twenty-four hour ECG (Holter monitor)
- Abdominal ultrasound scan
- Hearing test
You and your child may see a variety of members from the multidisciplinary team, these may include:
- A transplant nurse specialist
- A paediatric transplant consultant
- Doctors (junior doctors)
- Play specialist
- Social Worker
During your stay you and your child will also see a psychologist as part of the transplant assessment. This is because we recognise that undergoing a transplant can be an emotional and sometimes difficult experience and we want children and their families to be informed and fully prepared. Speaking to a psychologist may be a new experience for you and your child and it is something that you should not be worried about. Often the psychologist will speak to the parents and the child separately.
Before the operation
The waiting list
An up-to-date list of all paediatric patients awaiting transplantation is kept by the transplant team. We cannot predict how long your child will have to wait for a transplant – this is determined by availability of suitable donor organs and not by the length of time that your child has been waiting.
Selection of recipients (child waiting for a transplant) is based mainly on:
Specific factors affecting the suitability of a donor organ for your child - for example: blood group, height, results of antibody tests and tissue typing. Once your child is on the waiting list a suitable organ(s) may become available in just a few days but it can take many months or even years. During the waiting period your child will remain under the care of the referring consultant. However, we will request that you and your child return for reviews at GOSH. If your child's condition changes, new treatments are started, or if they deteriorate between visits, you and your child's referring doctor should keep us informed.
If there are any changes in your child's condition whilst they are on the waiting list the doctors may need to re-evaluate their suitability for a transplant and remove their name from the list, either temporarily (to allow for treatment), or permanently. If this happens you and your child will be informed and given an explanation, along with a follow up plan.
Unfortunately suitable organs do not become available in time for everybody who has been put on the waiting list. Some children therefore die whilst on the waiting list. This is a very sad reality that you, your child and your relatives should understand.
Coping with life on the waiting list
Undergoing a transplant is a time of great emotional and practical upheaval with many parents and children experiencing issues of concern. One particular area families can find difficult is the time on the waiting list. All families cope with life on the waiting list in very different ways. Here are some points that may help:
- Make plans about being called for a transplant from day one on the waiting list (it is never too soon to prepare).
- Please keep a bag packed and make clear plans with your family/friends to take care of other children.
- Remember life must go on as normal – make sure that daily activities/visits continue, while ensuring that you and your child are always contactable with appropriate travel plans in place. Upon listing, the transplant co-ordinator will discuss your specific travel needs with you.
- Take time to think through what transplantation means. If your child is old enough, it is important that you take the time to talk with them about what transplantation will mean to them. If you or your child have any concerns or require further information please contact the transplant team.
- We will need all home, school, work and mobile telephone numbers.
- If your child is admitted to another hospital, please ensure that the transplant co-ordinators are informed. Please let us know when your child is discharged.
- Keep the transplant nurses updated on major changes in your child's health.
Remember – whatever the problem, do not sit and worry about it. You can contact the transplant team for support and/or advice.
Admission for the transplant
When you receive the call from the transplant nurse co-ordinator, your child will need to travel to the hospital safely and in the shortest possible time. The transplant nurse co-ordinator will organise transport for your child. We advise that a maximum of two adults only can travel with your child. Transport will not be delayed to wait for a second accompanying adult. If your child is called to come in for transplant, these are the important things to remember:
- Do not allow your child to eat or drink from the time of the call, unless otherwise advised.
- Bring all of your child's current medication, including inhalers/nebulizers, and your own medications if you are on any.
- Bring all of your child's relevant respiratory/nutritional support equipment.
- Do not bring excess money or valuable belongings.
- Bring a bag for yourself and your child containing essential toiletry items, night clothes and light, comfortable shoes and clothing, and your child's favourite toys, DVDs, books and so on. It is a good idea to have this already packed in advance.
When you and your child arrive at the hospital
This is usually late evening or during the night as this is when most transplants take place. The co-ordinator will tell you on the telephone your child's admitting ward.
When you and your child arrive on the ward
You and your child will need to be as prepared as quickly as possible so that when the organ(s) arrive the surgeons can proceed with minimal delay. Your child's arrival on the ward and transfer to theatre can be rather hurried. Your child will be prepared and in the operating theatre for when the new organ(s) arrive. We hope that by notifying you and your child in advance of what to expect, you will feel less worried.
Given sufficient time many of the following may be carried out:
- Temperature, pulse and blood pressure readings and oxygen saturation
- Bacterial swabs
- Urine/sputum sample
- Weight and height measures
- Blood samples
- Chest x-ray
A doctor will examine your child and discuss any relevant or new medical problems. The doctor will discuss the operation again and then you and your child (where appropriate) will be asked to sign the consent form.
Your child will be seen by an anaesthetist and, if there is time, may be given a pre-medication to make them sleepy. Your child will also be started on their first dose of immunosuppression (anti-rejection) medications. Your child will continue to take immunosuppression medications after their operation and this will need to be continued for life.
Cancellation of your child's transplant operation
It is possible that when the surgeons arrive at the donor hospital, the organs are found to be unsuitable. We understand that this is extremely distressing and disappointing but unfortunately this will mean we will have to cancel the operation. You and your child should be aware that this might happen.
Although very rare, your child may have been anaesthetised by this time. You may find it helpful to discuss this with a member of the transplant team. If this decision is taken, it is only because we want to give your child an organ that has the best chance of functioning well.
Your child's transplant may also be cancelled if, on arrival at the hospital, your child has a high temperature or very abnormal blood results. Your child may then be kept in hospital for treatment, observation or reassessment.
The operation and afterwards
The team will start to prepare your child for the operation, if you feel comfortable you can accompany your child to the anaesthetic bay where your child will be anaesthetised and carefully monitored at all times. Once your child is asleep, you will be shown a comfortable place to wait for your child to return from theatre.
If you or your child would like to know more about the operative procedure, then please ask a member of the team.
Unexpected poor function of new organs
Although all organs to be transplanted are carefully checked by the team before transplant, in some cases following transplant the new organs do not perform as well as expected. There is not always a clear explanation for this. This can lead to serious complications, a prolonged stay in intensive care, and in some extreme cases, patients do not survive this period. The transplant nurse co-ordinator will keep you updated throughout your child's operation.
Depending on your child's transplant, and the surgeon, your child may have one of the following incisions:
- Sternotomy – an incision along the length of the sternum (breast bone)
- Clam shell – an incision below the breasts
- Thoracotomy – an incision around the side of the chest following the line of the ribs
After the operation your child will be transferred to intensive care unit. The staff will require approximately 30 to 60 minutes to settle your child into the intensive care unit, after this time you will be able to join your child.
Cardiac Intensive Care Unit (Flamingo Ward)
Intensive care can be a busy and daunting place, but the staff caring for your child are specially trained and will do everything they can to make sure your child is kept comfortable.
On your child's immediate return from theatre, they will be deeply asleep. As your child will be unable to breathe without help at this time, a breathing machine called a ventilator will take over your child's breathing until your child is more awake and able to breathe on their own. To connect your child to a ventilator there will be a tube, called an endotracheal tube, placed into your child's mouth (or nose if your child is very small) and down their windpipe. This will mean your child is unable to talk. Your child will also be attached to a heart and blood pressure monitor.
During the operation there is a risk of bleeding. There is a greater risk of bleeding if your child has had previous chest surgery. In the event of the bleeding being excessive after your child's operation, your child may need to be returned to the theatre for further surgery. All patients who have had a transplant will have a minimum of two chest drains on each side that will remove any blood and air that has collected at the site of the operation. These are removed when the bleeding has subsided. While your child is unable to eat and drink, food and fluids will be provided through a feeding tube or an intravenous (IV) infusion. This may continue until your child is able to eat and drink enough themselves. To assess your child's urine output, your child will have a urinary catheter in place that will drain urine from their bladder continuously.
If your child has undergone a heart-lung transplant, they may have 'pacing' wires through their chest, which may be connected to a 'pacing box'. These are temporary wires that may be used in the early stages following the transplant to keep your child's new heart beating regularly.
When your child's condition is considered stable, the medical team will reduce the sedation that is keeping your child asleep. This will allow your child to wake up and breathe for themselves. When your child is finally disconnected from the breathing machine, they will be able to speak and, in time, have a drink.
Children's' experiences of their time spent in intensive care can be varied and depend on the individual child and the length of their stay. Some children do experience vivid hallucinations which can be distressing to both you and your child. This is thought, in part, to be due to a withdrawal reaction from the medications that are given to keep children asleep and free from pain. In time, the hallucinations will reduce in both intensity and frequency. You and your child may wish to speak to someone if this happens.
The length of stay will vary from child to child from hours to days or even weeks, depending on their condition. As their stay in intensive care may be variable, you should prepare yourself for this.
Possible complications early after transplant
Poor kidney function and low urine output is common after transplant. This will usually resolve over a week or so. Sometimes kidney support is needed (haemofiltration) using a type of kidney machine. Infection is very common, but usually responds quickly to treatment.
Diaphragm palsy is caused by bruising and/or damage to the nerves and usually resolves over a few weeks. It does not tend to cause symptoms in older children, but young infants may require an operation to tighten the diaphragm (a breathing muscle located in the lower chest).
Hoarse voice is caused by bruising of the nerves / swelling of the windpipe and usually resolves over a few weeks.
Neurological problems such as fits/ convulsions/ headaches are common in the first week after transplant. Brain damage from the operation is rare and occurs in less than 1 per cent of transplant children. However, if a cardiac arrest occurs (the heart stops beating, requiring resuscitation) before or after the operation there is a risk of brain damage, which fortunately is rare.
Visiting is restricted for the first week after the transplant. It is important that you, your child and other relatives/friends understand this. For at least the first week in intensive care, visitors are limited to immediate next of kin, a maximum of two visitors at a time. To help stop the spread of infections, these need to be the same two visitors during this period. We do not recommend that babies and young children visit your child in intensive care. This is to protect your child, and them, from the risk of infection. All visitors are required to thoroughly wash their hands on entering and leaving the unit.
To further protect your child from infection, we advise that you do not bring any material (fluffy) toys and thoroughly clean any plastic toys before giving them to your child. It is helpful to have one key person who will inform the rest of your family/friends about your child's progress to avoid you and the nursing staff being disturbed by frequent telephone calls. Fresh flowers and plants are not allowed in intensive care, and are not recommended on the ward because of the risk of infection.
There are many ways to ensure that your child's pain is controlled, such as administration of intravenous (IV), epidural or oral medication. Your child's pain control requirements will be assessed and managed accordingly by the medical and nursing staff. Alongside medication management of pain control, there are also non-medical methods, which can be used in the control of your child's pain. These will often involve the play specialists.
The physiotherapist will see your child in intensive care to help them do breathing exercises, which will help maximise the function of their new lungs. When your child is transferred to Bear ward they will be encouraged to become progressively more mobile. We would like to return your child to an independent lifestyle as quickly as possible. Physiotherapy will involve techniques to help clear any sputum and get your child out of bed as soon as possible. If your child is of walking age, your child should be able to walk the length of the ward corridor and climb a flight of steps before discharge home.
When your child no longer requires intensive care, your child will be moved to Bear ward. While most of the monitoring equipment will have been removed, your child may still have intravenous infusions that were mentioned earlier. Over the following days, these will be gradually removed as your child's condition improves. You should play a role in encouraging your child to eat, drink and move about. If your child is old enough, you should also encourage them to gradually take care of their own needs.
Unlike intensive care, the nurse who is looking after your child will also have other patients to care for. You and your child may feel a little unsettled to begin with, but remember that your child would not have been moved to the ward unless their condition was progressing as expected.
If it is possible we would like you to be with your child regularly during their recovery. Accommodation can be provided for you – please speak to the transplant co-ordinators. You will be encouraged to participate in your child's care so that by the time your child is ready go home you will both feel more confident about what to do and how to avoid and/or manage complications.
The physiotherapists and nurses will help to mobilise your child and your child will become more independent. This is when you, and your child, if they are old enough, will start to learn about medications and about the main complications your child may experience post-transplantation, particularly rejection and infection.
Getting ready to go home
Everyone will respond differently after the transplant and this makes it difficult to predict how long your child will be in hospital, especially if your child was very unwell before the surgery. As soon as your child feels well enough, and staff are happy with your child's progress, your child's discharge date will be planned.
The transplant team will give you information throughout your child's stay. By the time your child is ready to go home. you (and your child, if they are old enough) should be able to manage medications prescribed. You should also be able to recognise the signs and symptoms of rejection and infection and know what action to take if these problems occur.
Your child will be taught how to use a hand-held spirometer. This is a device to measure your child's lung function, which they are advised to use on a daily basis, documenting the best of three blows. A 10 per cent drop in these readings for more than 24 hours may be a sign of rejection or infection and you are strongly advised to call the transplant team as soon as this occurs.
The physiotherapist will advise you on how your child should continue to improve their fitness and will ensure that your child's improvement in mobility is monitored safely, for example by climbing a flight of stairs.
Receiving a transplant will have already caused many changes in the life of you and your child. Your child may have altered expectations of themselves and may feel excited and happy, you will also likely have altered expectations of your child's abilities following the operation. However, on the other hand, you and your child may feel stressed, confused, even frightened. These are all perfectly normal feelings. It is important that you know that these feelings are normal and that there is someone from the team available to help, when you feel you want to talk.
During your child's admission the transplant team will be preparing you and your child for discharge home. The transplant team will answer any queries you may have about your child's follow-up. At this point you will be given your child's first outpatient appointment. You will also be given the transplant teams contact details.
Transplant flats are provided as a 'half-way house' for children and their parents who are not local but are well enough to be discharged from the hospital ward. The flats are subject to availability. All of our accommodation and flats are limited and always in high demand; however we try to meet all of our patients' needs as best we can. You may be asked to change accommodation from time to time. Although this may be unsettling we would appreciate your co-operation. The flats are strictly no smoking zones.
Transplant outpatients clinic
During the first six months, return visits for check-ups are frequent (weekly then reducing to fortnightly, monthly and so on) and will include lung function tests, x-rays and blood tests. If you live a long distance from the hospital you should think about how you will organise your child's visits. While you are getting used to medication strengths and dosages, it is advisable to bring all of your child's medicines with you for clinic appointments. Also always bring your child's medication diary when you come for any hospital appointment.
If you live a long distance from the hospital, overnight accommodation may be available, but priority is given to children and families on the ward. If your child complains of feeling unwell or if you are concerned in any way it is important that you contact us straight away as complications can be treated more easily if they are discovered early.
Although you are encouraged to contact the transplant team at any time with problems or worries, it is always preferable to contact the transplant team during office hours. However, do not delay in reporting symptoms – there is always someone at the hospital to give you advice 24 hours a day seven days a week.
Your child will be followed up for life by a transplant team. Eventually your child's routine visits will become much less frequent and you may be able to have blood tests done at your local hospital or family doctor (GP).
The clinic may be very busy and we aim to provide a quick and efficient service, however, occasionally delays do occur due to circumstances beyond our control. It is best to leave the whole day flexible when you and your child come to the clinic, especially in the first few months after your child's transplant. If your child is unwell, always ring and speak to a doctor or nurse before bringing your child to the clinic. By doing this, relevant tests can be organised in advance.
After transplant, your child will need regular blood tests to monitor for side-effects of the immunosuppressive therapy and to make sure that they maintain adequate immunosuppression to prevent rejection. Your child's medication doses may change following their blood tests.
Prior to your child's discharge you will be given a supply of your child's medication. Your child's GP and local pharmacist need to be informed of the medication your child is taking on discharge. A letter will be sent to your child's GP informing him of your child's medications, for future supplies. Most GPs will supply the medications for you, but a few decline in which case the local hospital will usually supply them.
It is important to consider how you will organise your own transport to and from hospital. Only in extreme circumstances the hospital will arrange transport to get you home, we need at least two days' notice to arrange this. You and your child's travel costs may be covered if you are receiving state benefits.