Nursing Care & Support

2015 review - A very good overview article most content is current today. Since the writing of this article advances in CLL understanding and research have resulted in advances in treatment and care. There are several new treatments involving next generation monoclonal antibody therapies and non chemo - BCR indibitors now availble for relapsed refractory, high risk and less fit patients. 


Liz Bishop, Nurse Consultant (Haemato-oncology), Guy's and St Thomas' NHS Foundation Trust


Chronic Lymphocytic Leukaemia (CLL) is the most frequent form of leukaemia and is more common in older people, the average age of patients at diagnosis being around 65-70 years.  Approximately, 20-30% of patients present under the age of 55 years old.  CLL is characterised by an excessive growth of cells (called B cells or B lymphocytes) in lymphoid tissues, bone marrow and the circulating blood.  The basic abnormality is the resistance of these mature cells to follow their natural programmed cell death (also called apoptosis).  Most patients (approximately 70-80%) are diagnosed by accident when being checked for another condition or routine health check.  This is usually noticed if the patient has a full blood count and the excess of B lymphocytes is found.  Other patients present with symptoms such as enlarged lymph nodes or lumps, tiredness, night sweats, weight loss or the symptoms of anaemia or infection. 

The clinical course of CLL is highly variable.  Typically it is very slow growing and many patients will have their CLL monitored at intervals on what we call a “watch and wait” programme, whereby they attend out-patient clinics for follow-up care.  Other patients, particularly those with symptoms or more rapid progression or associated complications, will require therapy.   Therefore, some patients live for decades with their CLL, whilst others experience clinical difficulties soon after diagnosis and require frequent and multiple forms of therapy.   Other patients may have been monitored for many years, learning to live with their CLL, but then require chemotherapy or develop complications such as infections.  This unpredictability can be difficult for patients and essentially they need different levels of care and support at different stages of their specific disease trajectory. 

What are the treatment options?

Treatment and care of these patients has remained relatively unchanged for many years but the good news is that research interest is growing in CLL with the recognition that new drugs and new regimes are producing higher complete remission rates and longer remissions..  Many patients of course require no therapy and are followed up carefully in the out-patient clinic under what is commonly known as a “Watch & Wait” programme.  If and when patients do require therapy there are several options available.  For years chlorambucil chemotherapy was the mainstay of therapy, however, other agents such as fludarabine and cyclophosphamide, rituximab, Campath, steroids and stem cell transplantation offer alternative treatment strategies.  In addition, the emergence of reduced intensity conditioning transplants also means that transplant may be an option for some older patients (see treatment).  Ideally nurses caring for CLL patients would be familiar with the complete range of therapies and have experience in administering chemotherapy and rituximab and Campath as well as experience of transplantation.  There is also increasing interest in the diagnosis and staging of CLL with the development of more accurate prognostic factors, for example, chromosomal changes (see prognostics ).  This is important because it may help to predict the clinical course of the CLL and therefore help with treatment planning. 

Why do patients need information and support?

This increasing complexity in both diagnosis and treatment means that patients have more information and support needs than before.   One could also argue that patients with CLL have always had specific information and support needs yet these have not always been recognised or developed within nursing to the same extent as for the more acute forms of leukaemia.  This is why the development of the CLL Support Association is important not only to ensure patients have a voice in their future but also to provide support for patients.   With increasing awareness of CLL among patients, clinicians and nurses and other healthcare professionals it is hoped that the support needs of CLL patients will be more fully met in the future.

Communicating the diagnosis to patients

Patients who are referred for a haematological opinion are entitled to be honestly informed about their disease and any subsequent management decisions in a sensitive way.  Patients may have to undergo a series of diagnostic tests, including blood tests, scans and possibly a bone marrow biopsy and will have to wait a few weeks until all the results are back.  Because CLL is characteristically a condition for which the natural history is measured in years, it is particularly important that patients are able to establish a relationship of trust with their clinician and hopefully a Clinical Nurse Specialist (CNS).   This can be an anxious time for patients but it is the beginning of a long-term patient-healthcare professional relationship. 

The word “leukaemia” itself can invoke fear and anxiety in the patient and the family.  They may assume they require immediate admission, chemotherapy and all its associated side effects and even a bone marrow transplant because generally speaking the population is not aware of the different types of leukaemia.  Of course, intensive therapy is not usually required at the outset, unless they are unwell at diagnosis and initially the patient will be followed closely in the out-patient clinic, without the immediate initiation of anti-leukaemia therapy.  Fortunately, anxiety can usually be prevented or at least reduced at the outset by a clear and careful description of the likelihood of it being slow growing.  However, the patient will also have to be informed of the variable clinical course of CLL, and therefore is faced with an unpredictable future.

 This inability to predict which clinical path the patient will follow presents a quandary for both patients and the haematology team.   Recent developments in prognostic testing have made it possible to predict which patients will have a more aggressive form of the disease. These tests are still research tools and have not yet made the transition into the clinical setting except as part of a trial.  As the understanding of prognostic factors improves it is anticipated patients will be able to have more information about the predicted course of their disease and these test results will help with treatment decisions. 

Who is the patient’s key worker?

The majority of patients benefit from, and should be offered, written information about CLL and its management, for example, that provided by the CLL Support Association.  Patients with any form of cancer, including CLL, should also be offered information on their team who is caring for them and be assigned a “key worker”. They should be informed who their key worker is and be provided with contact numbers.  This was specified in the government document ‘Improving Outcomes Guidance for Haematological Cancers’ published in 2003.  The key worker is usually a CNS but some hospitals may not have haematology CNSs and in these cases it is usually the Consultant who functions as the stable and constant resource for patients as the junior medical team rotate frequently.  In addition, many patients with CLL were diagnosed long before this key worker policy was developed and it remains a concern that these patients may have not been offered the opportunity of developing 1:1 relationships with a key worker in their nursing team.

Clinical Follow Up

It may be that patients remain anxious about their diagnosis of CLL for a considerable period of time.  It can take a while for confidence to return and gradually most can be reassured that it is a very slow growing condition, particularly as the gaps between their clinic visits are extended.  For others, the anxiety continues even in the absence of disease progression or associated problems.  For example, it is my own personal experience that there are patients who are reluctant to extend the gaps between clinic visits because they continue to be anxious about their CLL.  These patients may require reassurance and gradual extension between visits.  Building a trusting relationship with the patient usually helps as well as providing a list of names and telephone contact numbers, should they need help or telephone advice between clinic visits. 

Continuity of care and development of a close patient-healthcare professional relationship is an essential component of good quality CLL care.  The patient should always know who to contact should they develop any new problem plus the clinician and/or nurse should know his/her patients well enough to be able to detect subtle changes in their health as well as closely monitor their clinical condition.  This has the advantage that if and when therapy is initiated the contact and relationship is already established so that when the patient is at his/her most vulnerable they know how to access the system.

When to start therapy?

CLL presents a significant management problem because of the wide range of age of presentation, the natural history of the disease and also the frequency with which CLL is diagnosed in the pre-clinical stage (i.e. before there are any signs or symptoms).  However, it is important to note that the survival of CLL is superior to that seen in many other haematological malignancies and solid tumours.  Most patients with CLL will receive a number of different treatments during the course of the disease, but before treatment is initiated, other factors may have to be considered such as other health problems, general fitness and patient choice. (See  treatment).  Generally treatment is indicated if:

  • There is progressive bone marrow failure.  This is demonstrated by worsening blood counts, for example a fall in haemoglobin
  • Very large lymph nodes and/or spleen, especially if they are increasing in size rapidly  
  • Increasing lymphocyte count with a doubling time of less than 6 months
  • Systemic symptoms such as weight loss, fever, extreme tiredness and night sweats
  • Other complications of CLL such as auto-immune conditions(AIHA) may require treatment of the auto immune complications but not necessarily the CLL.

There is general agreement amongst clinicians on what the \indications are to start to treat. However research is being undertaken to establish whether patients who do need treating by conventional criteria, but who are at high risk of early progression, might benefit from pre-emptive treatment.  However, patients should feel reassured that most decisions to start treatment are now made in a Multi-Disciplinary Team (MDT) meeting.  The MDT meetings are regular planned meetings of consultant haematologists, pathologists, radiologists, oncologists, specialist nurses and junior medical staff to discuss the diagnosis and agree a management plan on every haematology cancer patient.  These are hospital-based meetings, which are now a government requirement since the “Improving Outcomes Guidance in Haematological Cancers” was published.  These are now well established in all hospitals.  Following a decision to treat CLL, the treatment plan then has to be discussed and agreed with the patient, documented in the medical case-notes and the GP informed. 

Undergoing Therapy

Some of the therapies are taken at home in the form of tablets for example chlorambucil, fludarabine and cyclophosphamide, whilst others require visits to the hospital day-care unit to receive intravenous therapy (a drip or injection into the vein).  Whatever the therapy of choice it is important the patient should receive information on:

  • The drug (s) and how to take the drug as well as the scheduling of the drugs
  • The potential side effects and how the patient should expect to feel
  • Practical information such as to how to monitor your temperature and look out for signs of infection or unusual side effects of therapy  
  • Contact names and numbers of specialist nurses to call during office hours and how to contact specialist medical advice during out of office hours.
  • Supportive medications to prevent side effects for example, anti-sickness drugs or antibiotics to prevent infection
  • Coping strategies

At times during periods of therapy or acute illness more intense contact with the team may be necessary.  The visits to the hospital may have to be more frequent.  Occasionally, other supportive therapy may be indicated such as blood transfusions or injections called growth factors, which help to boost the immune system.    Sometimes it is necessary to admit patients to hospital during these more intensive treatment periods or if complications occur such as severe infections.  Some of the regimens, for example, fludarabine combined with cyclophosphamide, are temporarily damaging to the immune system and therefore the patients need to be advised they are vulnerable to infection and should avoid obvious sources of infection as well as monitor their temperature.  The team should be able to give specific advice for each regime.

The Future

The future continues to look increasingly optimistic for CLL patients.  As can be seen from the CLLSA website ( New landscape & future section) there are a growing number of expert doctors and scientists with a passionate research interest in the diagnosis and management of CLL.  This has resulted in the pharmaceutical industry investing in anti-CLL therapies.

In summary, CLL is perhaps no longer the most forgotten leukaemia and there will be many future developments in the diagnosis and management of this disease.

Hopefully, patients will also become more informed in the management of their CLL, helping them to cope with their disease and make important decisions at different points in their cancer journey.