Born with cystic fibrosis, Sammi Sparke is now embarking on a new life thanks to an organ donor who gave her a new set of lungs, and her father’s donation of a kidney.

Before her lung transplant, Sammi, from St Neots, Cambridgeshire, was desperately ill and housebound, barely able to walk.

“I was not particularly conscious of my cystic fibrosis for most of my life, but in the three years before my lung transplant, I was very ill. I’d always had occasional intravenous antibiotics and spent time in hospital, but I never considered it as anything other than an obstacle to overcome,” says Sammi.

In 1998, Sammi contracted pneumonia after an operation on her lungs, and doctors told her she needed a lung transplant.

“I was shocked to learn from my consultant that I wasn’t going to get any better, as I’d always bounced back in the past. They said I should consider transplantation, and having to accept I was really ill made me depressed. I felt my life was over.

“I had some unusual medical problems, and for a time there was a question mark over whether they would attempt the transplant. I was so relieved when they said I could be registered for it. I had seen friends have transplants and do very well and seen other people with cystic fibrosis die because a donor was not found in time. It was a very scary time.”

Sammi had to give up her media studies degree in her second year as the disease left her housebound and almost helpless.

“I’d pass the time watching television and was inspired by travel programmes. I began to plan trips abroad for when I was well enough and started an Open University degree. Some days were better than others. I felt that all I had to cling to was the belief that one day I was going to receive the new lungs that would save me – that someone out there would be kind enough to donate them to me.”

A donor was eventually found to match Sammi’s needs, and a double lung transplant operation was performed at Papworth Hospital, Cambridgeshire, in August 2002.

“The difference after the operation was instantaneous. As soon as I woke up from the anaesthetic, I didn’t want to cough anymore. It was incredible. I could take a deep breath for the first time in years. I was still very weak from months of being housebound as my muscles had wasted away, but otherwise I felt very well and recovered from the operation quickly.”

Sammi enjoyed a new lease of life for two years following the transplant, but suffered a setback when her kidneys failed due to the anti-rejection medication she was taking. Sammi’s father stepped in to offer her one of his kidneys.

She has recently returned from a nine-month world trip, fulfilling the plans she made when waiting for her lung transplant.

Jon was diagnosed with cystic fibrosis as a baby and put on a lung transplant list aged 11. He talks about life before, during and after his transplant.

A lung transplant normally takes between four and 12 hours depending on the complexity of the operation.

After you have had your general anaesthetic, a breathing tube will be placed down your throat so your lungs can be ventilated.

Your chest will be opened and preparations made to remove the diseased lung or lungs.

If your circulation looks like it will need help, a cardiopulmonary bypass machine may be used to keep your blood circulating during the operation.

The old lung or lungs are removed and the new lung is sewn into place. When the transplant team is confident the new lung is working efficiently, the chest is closed and you will be taken off the bypass machine.

Tubes are left in the chest to drain any build-up of blood and fluid, and these will stay in place for several days.

You will be taken to the intensive care unit, where further tubes will be attached to supply your body with fluids and drugs and to drain urine from your bladder.

New surgical techniques

There are two new surgical techniques that will hopefully increase the number of donor lungs available for donation. These are described below.

Transplant after a non-heart beating donation

Most donations are taken from people who have died but whose heart is kept beating using life-support equipment – often these are people who have died after a long illness.

 

It is now possible to take lungs from a person who has died suddenly and then keep that lung “alive” for around an hour by passing oxygen into it. The oxygen keeps the biological processes of the lung going, which preserves the lung.

Ex vivo lung perfusion

Lungs can be damaged when the brain dies, before they are removed for donation. Because of this, only one in five lungs are suitable for donation.

Ex vivo lung perfusion is a new technique designed to overcome this problem. It involves removing the lungs from the body and placing them in a special piece of equipment known as a perfusion rig.

Blood, protein and nutrients are then pumped into the lungs, which repairs the damage.

The technique is still in its infancy, but hopefully it will eventually lead to an increase in the number of available donations.

If a lung transplant is thought to be an option for you, you will be referred for a transplant assessment.

Transplant assessment

You will need to stay in hospital for up to three days for a lung transplant assessment.

Tests are carried out to make sure your other major organs (such as your heart, kidneys and liver) will function properly after the transplant. These may include blood tests and any of the following investigations:

• chest X-ray
• echocardiogram, which checks how well your heart is pumping
• electrocardiogram (ECG), which records the electrical activity of your heart
• angiogram, an X-ray that takes pictures of the blood flow in the vessels of your lungs 

During the assessment, you will have the chance to meet members of the transplant team and ask questions. Your transplant team will include:

• surgeons
• anaesthetists
• intensive care specialists
• lung specialists
• specialists in infection
• a transplant nurse
• physiotherapists
• psychologists
• social workers
• a transplant co-ordinator

The transplant co-ordinator (your main point of contact) will talk to you and your family about what happens, and the risks involved in a lung transplant.

When the assessment is complete, it will be decided whether a lung transplant is suitable for you and if it is the best option.

It may be decided that:

• you should go on the active waiting list – which means you could be called for a transplant at any time
• a transplant is suitable for you, but your condition is not severe enough – you will be reviewed regularly and if your condition worsens, you will then be put on the active waiting list
• you need more investigations or treatment before a decision can be reached
• a transplant is not suitable for you – in this case, the assessment team will explain why and offer you other options, such as drug therapy or alternative surgery
• you need a second opinion from a different transplant centre

Why you might be unsuitable for a lung transplant

The supply of donor lungs is limited, which means there are more people who would benefit from a transplant than there are donor lungs.

This means people who are unlikely to have a successful transplant are not usually considered suitable for transplant.

You may be considered unsuitable if:

• you have not complied with previous advice or been reliable – for example you have not given up smoking, you have a poor history of taking prescribed medication or you have missed hospital appointments
• your other organs, such as your liver, heart or kidneys, do not function well and, therefore, may fail after the stresses of the transplant operation
• your lung disease is too advanced, so it is thought you would be too weak to survive surgery
• you have a recent history of cancer – there is a chance that the cancer could spread into the donated lungs; exceptions can be made for some types of skin cancer as these are unlikely to spread
• you are carrying an infection that would make the transplant too dangerous
• you have psychological and social problems that may affect whether you take post-transplant treatments; such as being addicted to drugs or having a serious mental health condition
• you are significantly underweight with a body mass index of less than 16 or overweight (obese) with a body mass index of 30 or above

Age also plays a part due to the effect it has on likely survival rates. There are no set rules and exceptions can always be made, but as a general rule:

• people over the age of 50 would not be considered suitable for a heart-lung transplant
• people over the age of 65 would not be considered suitable for a single or double lung transplant

The waiting list

Once you are on the active waiting list, the transplant centre may give you a pager so you can be contacted at short notice. 

The length of time you will have to wait will depend on your blood group, donor availability and how many other patients are on the list (and how urgent their cases are).

While you wait, you will be cared for by the doctor who referred you to the transplant centre. Your doctor will keep the transplant team updated with changes to your condition. Sometimes, another assessment is necessary to make sure you are still suitable for transplant.

Generally, your transplant team will be given relatively short notice of donor organs, so will have to move swiftly. When a suitable donor is found, you will normally be in hospital ready for your transplant within six to eight hours. If you live a long way from a transplant centre, you will be flown to the centre or taken by ambulance.

Getting the call

When a suitable donor lung is found, the transplant centre will contact you and ask you to go to the centre.

When you hear from the transplant centre:

• do not eat or drink anything
• take all current medicines with you
• take a bag of clothes and essentials for your stay in hospital

At the transplant centre, you will be reassessed quickly to make sure no new medical conditions have developed. At the same time, a second medical team will examine the donor lungs.

The procedure must be carried out as quickly as possible for the transplant to have the best chance of success.

A lung transplant is an operation to remove and replace a diseased lung with a healthy human lung from a donor. A donor is usually a person who has died, but in some cases a section of lung can be taken from a living donor.

Lung transplants are not carried out frequently in the UK; mainly due to the lack of available donors. From April 2013 to April 2014 there were 179 lung transplants performed in England.

A lung transplant is used to treat:

• people with advanced lung disease who are failing to respond to other treatment
• a person whose life expectancy is thought to be less than two to three years without a transplant

Conditions that can be treated with a lung transplant include:

• chronic obstructive pulmonary disease (COPD) – a general term that refers to a number of diseases that damage the lungs, most commonly as a result of smoking
• cystic fibrosis – a genetic condition that causes the lungs and digestive system to become clogged up with a thick sticky mucus
• pulmonary hypertension – high blood pressure inside the vessels that carry blood from the heart to the lungs
• idiopathic pulmonary fibrosis – scarring of the lungs

Types of transplant

There are three main types of lung transplant:

• a single lung transplant – where a single damaged lung is removed from the recipient and replaced with a lung from the donor; this is often used to treat pulmonary fibrosis but is not suitable for people with cystic fibrosis as infection will spread from the remaining lung to the donated lung
• a double lung transplant – where both lungs are removed and replaced with two donated lungs; this is usually the treatment of choice for people with cystic fibrosis or COPD
• a heart-lung transplant – where the set of lungs and the heart is removed and replaced with donated heart and lungs; this is often recommended for people with severe pulmonary hypertension

Read more detailed information about a heart-lung transplant.

Will I be able to have a lung transplant?

The demand for lung transplants far outstrips the available supply. So a transplant will only be carried out if it is thought there is a relatively good chance of it being successful.

For example, a lung transplant would not be recommended for people with lung cancer as the cancer could reoccur in the donated lungs.

You will not be considered for a lung transplant if you currently smoke.

Living donors

Having a lung transplant from a living donor is sometimes possible.

Two living donors are usually required for one recipient. The lower lobe of the right lung is removed from one donor and the lower lobe of the left lung is removed from the other donor. Both lungs are then removed from the recipient and are replaced by the lung implants from the donors in a single operation.

Most people who receive lung transplants from living donors have cystic fibrosis. Most are close relatives of the recipients. The donors and the recipient need to be compatible in size and have matching blood groups.

Getting ready

Before being placed on the transplant list you will need a series of tests to make sure your other major organs (such as the heart, kidneys and liver) will function properly after the transplant.

It is also important to make lifestyle changes to get as healthy as possible when the time comes for the transplant to take place.

Read more about preparing for a lung transplant.

What happens during a lung transplant?

A lung transplant normally takes between four and 12 hours to complete, depending on the complexity of the operation.

A cut is made in your chest and the damaged lungs removed. Depending on your individual circumstances you may be connected to a bypass machine (heart and lung machine) to keep your blood circulating during the operation.

The donated lungs are then connected to the relevant airways and blood vessels and the cut is closed.

Read more about how a lung transplant is performed.

Risks

A lung transplant is a complex type of surgery and carries a high risk of complications.

A common complication is the immune system rejecting the donated lungs. Because of this, a type of medication called an immunosuppressive is given to dampen the effects of the immune system, reducing the risk of rejection. However, taking immunosuppressives carries its own risks as they make a person more vulnerable to infection.

Read more about risks associated with a lung transplant.

Recovery

A lung transplant is a major operation that may take at least three months to recover from.

It could be quite a while before you are able to return to work so you will need to make necessary arrangements with your employer.

Read more about recovering from a lung transplant.

Outlook

The outlook for lung transplants has improved in recent years and is expected to continue to improve in the future.

The British Transplantation Society estimates that around nine out of 10 people will survive for at least a year after a transplant and five out of 10 will survive for at least five years (there have been reports of people living for 20 years or more after a transplant).

Though complications can occur at any time, a serious complication is most likely to occur in the first year after the transplant.

After surgery, you will remain in the intensive care unit for around one to seven days. You may have an epidural (a type of local anaesthetic) for pain relief and will be connected to a ventilator to help your breathing.

You will be carefully monitored so the transplant team can check your body is accepting the new organ. This monitoring will include regular lung X-rays and lung biopsies (where tissue samples are taken).

The transplant team can determine whether your body is rejecting the lung from the biopsy results. If it is, additional treatment will be given to reverse the process.

When your condition is stable, you will be moved to a high-dependency ward, where you will stay for one or two weeks.

Follow-up appointments

You will probably be discharged from hospital two to three weeks after surgery and asked to stay near the transplant centre for one month.

For the second month, you will need to visit weekly for four weeks. After that, for the rest of your life, you will have a blood test every six weeks and will be seen at the transplant centre every three months.

Getting back to normal

It normally takes at least three to six months to fully recover from transplant surgery. For the first six weeks after surgery, avoid pushing, pulling or lifting anything heavy. You will be encouraged to take part in a rehabilitation programme involving exercises to build up your strength.

You should be able to drive again four to six weeks after your transplant, once your chest wound has healed and you feel well enough.

Depending on the type of job you do, you will be able to return to work around three months after surgery.

Immunosuppressant therapy

You will need to take immunosuppressant medications, which weaken your immune system so your body does not try to reject the new organ.

There are usually two stages in immunosuppressant therapy:

• induction therapy – where you are given a combination of high dose immunosuppressants immediately after the transplant to weaken your immune system; you may also be given antibiotics and antivirals to prevent infection
• maintenance therapy – where you are given a combination of immunosuppressants at a lower dose to ‘maintain’ your weakened immune system

You will need to be treated with maintenance therapy for the rest of your life.

Most transplant centres use the following combination of immunosuppressants:

• tacrolimus 
• mycophenolate mofetil
• corticosteroids 

The downside of taking immunosuppressants is that they can cause a wide range of side effects, including:

• mood changes such as depression or anxiety
• insomnia
• diarrhoea 
• swollen gums
• bruising or bleeding more easily
• convulsions
• dizziness
• headache 
• acne 
• extra hair growth 
• weight gain

Your doctor will try to find the right dose that is high enough to ‘dampen’ the immune system, but low enough that you experience few side effects. This may take several months to achieve.

Even if your side effects become troublesome, never suddenly stop taking your medication because your lungs could be rejected.

Long-term use of immunosuppressants also increases your risk of developing other health conditions such as kidney disease – read more about the risks associated with long-term immunosuppressants use.

Avoiding infection

Having a weakened immune system is known as being immunocompromised. If you are immunocompromised, you will need to take extra precautions against infection. You should:

• practise good personal hygiene – take daily baths or showers and make sure that clothes, towels and bed linen are washed regularly
• avoid contact with people with infections that could seriously affect you – such as chickenpox or influenza (flu)
• wash your hands regularly with soap and hot water – particularly after going to the toilet and before preparing food and eating meals
• take extra care not to cut or graze your skin – if you do, clean the area thoroughly with warm water, dry it, then cover it with a sterile dressing
• keep up to date with regular immunisations – your transplant centre will supply you with all the relevant details

Be aware of any initial signs that you may have an infection. A minor infection could quickly turn into a major one.

Tell your GP or transplant centre immediately if you have symptoms of an infection. These include:

• fever (high temperature) of 38C (100.4F) or above
• headache
• aching muscles

A lung transplant is a complex operation and the risk of complications is high.

Some complications are related to the operation itself. Others are a result of immunosuppressive medication, which is needed to prevent your body rejecting the new lungs.

Some of these complications are discussed below.

Reimplantation response

Reimplantation response is a common complication affecting almost all people with a lung transplant. The effects of surgery and interruption to blood supply causes the lungs to fill with fluid.

Symptoms include:

• coughing up blood
• shortness of breath
• difficulties breathing while lying down

Symptoms are usually at their worst five days after the transplant. These problems will gradually improve, and most people are free of their symptoms by 10 days after their transplant.

Rejection

Rejection is a normal reaction of the body. When a new organ is transplanted, your body’s immune system sees it as a threat and produces antibodies against it, which can stop it working properly. Most people experience rejection, usually during the first three months after the transplant.

Shortness of breath, fatigue (feeling tired all the time), and a dry cough are all signs of rejection, although mild cases may not always cause symptoms.   

Acute rejection usually responds well to treatment with steroid medication.

Bronchiolitis obliterans syndrome

Bronchiolitis obliterans syndrome (BOS) is another form of rejection that typically occurs in the first year after the transplant, but could occur up to a decade later.

In BOS, the immune system causes the airways inside the lungs to become inflamed, which blocks the flow of oxygen through the lungs.

Symptoms include:

• shortness of breath
• dry cough
• wheezing

BOS may be treated by giving you additional immunosuppressant medications.

Post-transplantation lymphoproliferative disorder

Post-transplantation lymphoproliferative disorders (PTLD) are thought to affect around one in 20 people after a lung transplant.

PTLD is thought to occur when a type of viral infection stimulates abnormally high production of B-cells. This would normally be controlled by T-cells, but the immunosuppressants block the effects of T-cells. 

Treatment options will depend on the type of PTLD and where in the body it is situated.

Infection

The risk of infection for people who have received a lung transplant is higher than average for a number of reasons, including:

• immunosuppressants weakening the immune system, meaning an infection is more likely to take hold and a minor infection is more likely to progress to a major infection
• people often have an impaired cough reflex after a transplant meaning that they are unable to clear mucus from their lungs – providing the perfect environment for infection
• surgery can damage the lymphatic system, which usually protects against infection
• people may be resistant to one or more antibiotics as a consequence of their condition, especially those with cystic fibrosis

Common infections after a transplant include:

• bacterial or viral pneumonia
• cytomegalovirus (CMV) 
• aspergillosis – a type of fungal infection caused by spores

Long term use of immunosuppressants

Taking immunosuppressant medications is necessary following any type of transplant, though they do increase your risk of developing other health conditions.

These health conditions are described below:

Kidney disease

Kidney disease is a common long-term complication. It is estimated that one in four people who receive a lung transplant will develop some degree of kidney disease a year after the transplant.

About one in 14 people will experience kidney failure within a year of their transplant, rising to one in 10 after five years.

Diabetes

Diabetes, specifically type 2 diabetes, develops in around one in four people a year after the transplant.

Diabetes is treated using a combination of:

• lifestyle changes, such as taking regular exercise
• medication, such as metformin or injections of insulin

High blood pressure

High blood pressure develops in around half of all people a year after a lung transplant and in eight out of 10 people after five years.

High blood pressure can develop due to a side effect of immunosuppressants or as a complication of kidney disease.

Like diabetes, high blood pressure is treated using a combination of lifestyle changes and medication.

Osteoporosis

Osteoporosis (weakening of the bones) usually arises as a side-effect of immunosuppressant use.

Treatment options for osteoporosis include vitamin D supplements (which help strengthen bones) and a type of medication known as bisphosphonates, which help maintain bone density.

Cancers

People who have received a lung transplant have an increased risk of developing cancer at a later date. This would usually be one of the following:

• skin cancer 
• lung cancer 
• liver cancer 
• kidney cancer 
• non-Hodgkin lymphoma – which is a cancer of the lymphatic system

Because of this increased risk, regular check-ups for these sorts of cancers may be recommended.