De Soyza AG, Corris PA. Feb, 2002. Transplantation [online]. Freeman Hospital, Newcastle Upon Tyne, UK. Available from http://www.cysticfibrosismedicine
Since 1985 lung transplantation has become an option for some patients with cystic fibrosis and end stage lung disease. It offers improvement in quality of life and increases life expectancy. It is important to note that CF patients experience some of the best post-transplant survival rates as compared to other lung transplant indications. There is a trend towards improved survival with time; a number of centres now have recipients who are 10-year survivors. To date post transplant survival is around 84% at 1 year, 50% at 5 years and 38% at 8 years. Mean FEV1 increases from around 20-25% pre-transplant to 79% one-year post transplant. While the majority of patients are able to return to normal activity there are many potentially serious complications that can occur in both the peri-operative and late post-transplant periods.
The introduction of transplantation as a treatment option can be a distressing experience for patients and their families. Individuals often have a range of feelings including, shock, denial and anger. Patients and relatives often want to know "are we really at this point?", "do we really have to rush into this?", "how much time do we have to decide?" At this stage, individuals must be given plenty of opportunity to discuss their thoughts and feelings with various members of the team.
Once the decision has been made to proceed with the transplant assessment, then a referral is made to a transplant centre. Individuals being referred will have already been through some sort of assessment at their local CF centre.
For the assessment, patients and their families stay for approximately four days at the transplant hospital, where accommodation is provided. Here both patient and carer(s) meet the transplant team and have the opportunity to ask questions and discuss any concerns. The patient undergoes a series of tests to ensure that transplantation is appropriate. The results of these tests are then discussed and one of three options is decided upon:
We follow current guidelines for pulmonary transplantation. Our assessment procedure is conducted as an in-patient over 4 days with assessment of physical, functional and psychosocial parameters.
Pulmonary function and arterial blood gases
Pulmonary function is assessed according to BTS guidelines. We routinely assess Forced Expiratory Volume 1 second (FEV1) and Forced Vital Capacity (FVC) by spirometry (Vitalograph, Bucks, UK). Bronchodilator reversibility testing is also undertaken. Arterial blood gases are routinely performed on air unless the patient was oxygen dependent.
Renal and biochemical assessment
Routine biochemistry including electrolytes, urea, creatinine, alanine transferase; alkaline phosphatase bilirubin and C-reactive protein are recorded.
Radio-isotope studies
Assessment of renal function was performed using the radiolabelled EDTA glomerular filtration rate technique (51EDTA GFR studies). Liver imaging, using radioisotope 99Technetium liver scans or ultrasound is also performed.
Haematological assessment
Routine haematology assessment of Haemoglobin (Hb), White cells (WCC) and platelets, prothrombin time (PT) and activated partial thromboplastin time (APTT)is undertaken. The coagulation screen helps to define any hepatic dysfunction.
Bone mineral densitometry
Bone densitometry is performed on lumbar vertebrae and the femoral neck using a densitometer. We use a modification of the WHO criteria for osteoporosis and osteopenia.
Exercise assessment
Functional assessment using the six-minute walk (SMW) test is performed where possible for each patient in an enclosed corridor over a 50-metre length. Oxygen saturation at rest and throughout exercise was recorded using a handheld oximeter. The lowest desaturation during or within the rest period at the end of the test is recorded.
Social assessment
Caring networks and support are explored by the transplant social workers. Feelings and anxieties towards transplantation are discussed with the patient and coping mechanisms are also discussed. An assessment of the social benefits and financial position of the patient and family is also undertaken to ensure all appropriate financial support is in place.
Result of transplant assessment
Patient provisionally accepted: The patient is a suitable candidate for transplant but is too well at the present time. In this case an appointment to see the transplant team will be arranged for a few months later.
Patient actively accepted: If the patient is put on the active waiting list, more detailed information about the next stage is provided by the transplant team.
Patient not accepted: Whilst this seldom happens, sometimes patients are not considered to be suitable for transplantation for medical reasons. These may be temporary or permanent e.g. infected indwelling IV device and severe kyphoscoliosis. It is estimated that between 4% and 10% of candidates are not suitable and it is crucial that these individuals are offered emotional support.
Patient declines transplantation: Post transplant lifestyle remains far from normality with post-transplant bronchoscopies and lifelong anti-rejection therapy areas of concern for patients. There is a small minority that are reluctant to overcome such concerns and decline transplantation. Alternatively some patients accept they may require a transplant but do not feel it "the right time for them". Such patients are therefore offered early review by the transplant team.
The waiting period can last from a few days to over three years. At this stage patients must be contactable at all times and are usually given a 'bleep'. The uncertainty of this phase can again, be very stressful for patients and families. Further psychosocial assessment takes place at regular intervals during this phase and rapid access to support is made available. Common emotional problems arise from avoiding infections (isolation), increases in treatment (which can be time consuming and exhausting), maintaining meaningful social ties (with family and friends), dealing with jealousy ("why did x get called before me?") and keeping hope alive.
Patients and their relatives often remember in considerable detail where they were and what they were doing when the call came for transplant (even in the case of false alarms). Later, if this imagery were to become distressing, brief psychological work has been shown to be effective.
What type of transplant operations are there?
There are four distinct operation types:
Single lung transplantation
This is the most commonly performed lung transplant operation worldwide. The operation is performed via thoracotomy and involves transplanting a single donor lung into the ipsilateral chest of a recipient. Single lung transplantation is carried out for end stage non-septic lung disease such as emphysema, sarcoidosis and idiopathic pulmonary fibrosis. It is not performed for septic lung conditions such as cystic fibrosis because the remaining infected native lung can cause contra-lateral lung as well as generalised sepsis.
Sequential single lung transplantation
This operation is usually carried out via a modified bilateral thoracotomy with lower sternal division called a clamshell incision. During the procedure both donor lungs are transplanted sequentially into the recipient. The clamshell incision provides excellent exposure of the thoracic cavity, making it easier to tackle chronic pleural adhesions. Sequential single lung transplant operation is performed for both non-septic and septic pulmonary conditions (such as cystic fibrosis) as long as there is reasonable cardiac function. The bilateral sequential lung transplant procedure has the advantage of leaving the patient with his/her own heart. It carries similar survival figures to heart/lung transplantation.
Heart lung transplantation
The operation involves transplanting both donor heart and lungs into a recipient. Some centres perform a domino transplant procedure where a recipient receives a heart lung transplant and their explanted heart is then used for a heart transplant candidate. A potential disadvantage of this operation is that the donor heart may be predisposed to chronic rejection related coronary heart disease. The operation is performed for conditions such as congenital heart disease with Eisenmengers syndrome, primary pulmonary hypertension and cystic fibrosis patients.
Lobar donation and living related lobar transplantation
Lobar donation is usually performed in children and small adults with end stage respiratory disease. Severe sepsis is often a contraindication as the frequent size mismatch between donor lobe and recipient's thoracic cavity predisposes to post operative sepsis. Size mismatch can also prolong the need for chest drains.
In living related lobar transplantation, the lower lobes from two healthy blood relatives are transplanted into a recipient. The procedure carries similar survival figures to sequential single lung transplantation. There are however important ethical dilemmas about the appropriateness of living related transplantation in view of the small donor morbidity and the potential risk of donor mortality. Cadaveric lobar donation At operation the donor lungs are split into lobes. The lower lobes are then transplanted into the recipient in a similar fashion to the above. I general this is only satisfactory for paediatric or small stature adults.
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