URGENT: A father of 7, recently diagnosed with MDS needs your help

Your animated guide to becoming a bone marrow donor. Find out everything you ever wanted to know about donation, from joining the register to what happens if you are a match.

A part of your bones called “bone marrow” makes blood cells. Marrow is the soft, spongy tissue inside bones. It contains cells called “hematopoietic” stem cells (pronounced he-mah-tuh-poy-ET-ick). These cells can turn into several other types of cells. They can turn into more bone marrow cells. Or they can turn into any type of blood cell. Here is a short educational film by the Irish Stem Cell Foundation

Suffering from MDS causes a steady and persistent decrease in the functional ability of patients partially due to fatigue and this has a negative impact on QoL in a number of ways. Fatigue experienced in MDS patients is as a result of low haemoglobin levels due to anaemia and it could have an impact on the physical aspects of a patient’s life(2). The physical aspects of fatigue experienced by MDS patients is often associated with decreased strength, unusual tiredness and weakness, lack of energy and the persistent need to sleep or rest. This fatigue is usually not as a result of a recent strenuous activity and could affect the functional and cognitive ability of a patient. This can lead to early retirement or reduction in work hours for patients who are not of retirement age thus resulting in financial consequences for the patient and their family. Similarly, it can lead to restriction in daily activities, planning for the future and diminished independence for patients who are of retirement age.
Based on QoL-e scores(3), the majority of patients found it very difficult performing heavy activities and experienced added fatigue simply when carrying out daily activities.
When asked about the greatest challenge of having MDS, patients said:

'Chronic fatigue affects my day to day activities'
'I look too well to be ill and it is hard to explain the deep fatigue I feel'
'The fatigue and how it can impact on daily life some of the time. Finding it difficult to explain to people that the tiredness I feel is not the sort one might have in life in general'
'Sudden change in lifestyle from being fit and healthy to being restricted by fatigue and hospital visits'

Use of red blood cell (RBC) transfusions as a form of supportive care

Supportive care with red blood cell transfusions is usually the primary and standard management strategy particularly for patients with low-risk MDS based on the Revised International Prognostic Scoring System (IPSS-R)(4). MDS patients are usually anaemic at the time of their diagnosis and would rely on RBC transfusions to correct the symptoms at some point in their MDS journey.
Based on our survey:

62% received transfusions as part of their MDS treatment.
59% of those who received transfusions had red blood cell transfusion as part of their MDS treatment.

How often RBC transfusion is given varies between patients; some need them as often as every week while others need regular transfusions every couple of months. Likewise, pattern or frequency of transfusion can change over the course of the disease. In the UK, patients are usually transfused when their haemoglobin level drops to 80 to 85 grams per litre of blood(5). However once a patient is transfused, the level of haemoglobin does not remain constant and is not long-lasting. Haemoglobin levels start to drop after being transfused thus the symptoms of anaemia resurfaces before the next transfusion is due. This leads to fluctuation in the energy levels of the patient hence affecting QoL. Based on our survey, red blood cell transfusion dependence (RBC-TD) at the time of the survey correlates with decreased or compromised abilities compared to red blood cell transfusion free (RBC-TF) patients (See graph below).

There are no set haemoglobin concentrations at which blood is transfused at. It is based on different individuals; this level will differ between patients depending on the presence of co- morbidities such as heart conditions(6). Therefore, haematologists need to assess the symptoms of a patient before deciding what level they should be transfused at. Also, new research is going on to assess whether MDS patients should be transfused restrictively or in a more liberal manner. It also aims to investigate whether transfusions at more frequent intervals could potentially reduce fluctuations in energy levels therefore improving QoL(7). Visit our page to get more information on a recently concluded trial (REDDS trial) which investigates this: https://mdspatientsupport.org.uk/redds-clinical-trial
The negative impact of MDS on a patient’s QoL can worsen as a result of the added efforts spent trying to cope with the disease such as repeated hospital visits for transfusions, waiting times as well as managing the side effects of transfusions.

RBC-TD patients will be on transfusions for a significant amount of time. At time of survey, 27% RBC-TD patients had received transfusions for 2 years or more.
According to our survey, the majority of RBC-TD patients are on a transfusion pattern of 2 - 4 weeks.

These efforts could potentially use up the limited energy stores of a patient thus affecting their QoL. Regular visits to the hospital for transfusion can have a financial impact on households.

31% and 21% RBC-TD patients respectively reported that cost for hospital travel and car parking had an impact on their household budget.

Also, reliance on family members or friends for support can affect a patient’s independence thus resulting in additional concern and stress for some patients and their families. This is worse for patients who don’t benefit from any support network. Based on our survey:

81% RBC-TD patients required support as a result of having MDS.
59% RBC-TD patients relied on their spouse for transport to medical appointments.

Apart from the physical health issues caused by MDS, emotional impact of the disease can be problematic as a result of uncertainties associated with the disease. RBC-TD patients worried about MDS to a significant extent compared to RBC-TF patients and suffered a higher emotional burden (See graphs below). QoL-E scores(8) were significantly lower (worse QoL) in RBC-TD patients in all domains (physical, functional, social, sexual and emotional wellbeing).

Transfusion dependent MDS patients have been found to have a lower QoL compared to transfusion free patients(9). Our survey also supports this statement; QoL-E scores were found to be significantly lower (worse QoL) in RBC-TD patients in all domains. This could be due to the fluctuations in haemoglobin and energy levels, transfusion reactions, infections or accumulation of iron in the body.
Based on the MDS UK survey:

15% of RBC-TD patients had complications or side effects from transfusions.
44% of RBC-TD patients required hospitalisation due to the side effects.

Patients who are on long-term RBC transfusion are inevitably susceptible to developing iron overload. Increased amount of iron in the body results in the build-up of iron in various organs, in particular, the heart, liver, kidneys and endocrine organs thus leading to the continual deterioration of these organs. There are tests available to assess iron overload in a patient; the most common is a blood test called a Ferritin test. Iron chelation therapy is required to remove the excess iron in these patients and it is administered either via the Deferoxamine pump or oral tablet, Deferasirox (Exjade). Based on our survey:

24% of RBC-TD patients received iron chelation therapy.

Though iron chelators are administered to patients with iron overload, they can have side effects which can be challenging for patients. Common side effects are: body rash or hives, digestive problems, anaphylactic shock, swelling or lumps at the local infusion site to mention a few(10). Also, using the Deferoxamine pump can be inconvenient and uncomfortable for patients as they have to insert a needle into the stomach and wear the pump overnight. This can disrupt a patient’s sleep thus affecting their limited energy levels. Most side effects can be prevented or managed by working closely with the clinical staff. Discuss any symptoms you have developed after starting iron chelation therapy with your clinical team.

Below is a word cloud illustrating the most quoted words when patients were asked "What do you found to be the greatest challenge of your treatment for iron overload?"

QoL-e results relating to physical well-being matched these findings from the general part of our survey. As a result, patients have to navigate the health system, speak to different consultants and manage multiple health visits sometimes in different hospitals. In some cases, patients are forced to ensure that there is communication between the consultants in the same and/or different hospital departments. As a result, there is a need for physicians treating the other health issues to liaise and collaborate with haematologists in charge of MDS care. Thus, an overall treatment plan adjusted for other health issues can be developed for the patient.

Summary

The consequences of MDS can be debilitating thus affecting the physical abilities of a patient. Additional factors associated with multiple disease management such as side effects from treatments, travel time and regular hospital visits by MDS patients can further impact QoL. Therefore, it is important to offer these patients some increased support and a more coordinated/collaborative care so as to improve QoL.

The impact of MDS on emotional well-being can be challenging. This is usually due to uncertainties which can arise as a result of inadequate understanding of the disease, fear of potential disease progression therefore leading to anxiety and worry. Unfortunately, care and attention is sometimes too focused on patients physical problems associated with MDS. This is where the role of an MDS nurse and counsellor lies. The former helps patients understand MDS better, identify and assess any physical issues associated with MDS as well as help navigate through the complex health care system. The latter helps patients understand, reflect on and work through concerns and worries they may have thus potentially improving their QoL. Patient support groups are also an excellent source of reassurance for MDS patients.

Recommendations

RAF Coningsby is an iconic location being the original home of ‘The Dambusters’. It’s currently home to a squadron of Typhoons amongst other aircraft.

RAF Coningsby was chosen because Adrian is a pilot with the RAF, and the RAF had been instrumental in supporting a bone marrow donor drive. We were lucky enough to have a great group of supporters on the day. These included Tom Lightfoot, himself an MDS patient who’s had a successful transplant, Jayne Snell, another MDS patient who’s also had a successful transplant, together with her husband, Martin, and Russell Cook, deputy chairman of MDS UK, there to represent the charity in an official capacity.

It was a long day - with some of our supporters driving 2 to 3 hours or more in order to get to the event - but well worth it. The support from the RAF was spectacular with over 75 RAF and support personnel swabbed ready to be put onto the bone marrow registry - and the general consensus on the day was that we could probably do this again with even greater effect.

A surprise on the day was a radio interview for Russell during which he managed to ad lib his way through a 10 minute phone call, live on air, with Melvyn Prior of BBC Radio Lincolnshire! During the interview he emphasised how important it was for people to come forward as donors, that although the event was restricted to base personnel, that the public could join the bone marrow register by contacting the charities directly and asking for either a swab or spit kit. He also highlighted the problems that a great many MDS patients suffer with; namely, fatigue, nausea, bleeding and infections - and pointed out that a great many GPs will never have met a patient with MDS.

Although only 1 in 10 MDS patients will be eligible and fit enough to tolerate a bone marrow transplant it goes without saying that adding to the registry is absolutely vital. At any one moment in time there are circa 1600 people with blood cancer in the UK in desperate need of a bone marrow match but for whom nobody has as yet come forward.

Currently, only 69% of all blood cancer patients can find the best possible match from a stranger, and this drops dramatically to 20% if you're a patient from a black, Asian or ethnic minority background.

By building and diversifying the register we will be able to provide the best match to even more people with blood cancer. Somewhat shockingly only 2% of the population of the UK are registered as potential donors compared to 9% in Germany. This goes a long way to explain why somewhere in the region of 3 out of 5 donors to UK patients are from Germany. These events will hopefully add to the pool of resources that cancer patients in the UK are reliant on.

We hope that by highlighting MDS as a hidden and rare blood cancer that we will be able to garner further support either in person or financially so that we can help fund research into the disease.

Current research

This exciting discovery has led to the testing of vitamin C in patients with MDS to see if it can improve current treatment with azacytidine. There is certainly evidence to suggest that vitamin C can enhance the effects of azacytidine but we don’t know yet whether this will work similarly in MDS patients. Nor do we know the dose of vitamin C that patients would need to take or even whether vitamin C is at all harmful at higher doses. That is why several clinical trials, both in Europe and the USA, are now looking at whether vitamin C can be a useful treatment in MDS.

First of all we have to establish that vitamin C is safe for patients and does not have unwanted side-effects.

Once we have found out the best dose, then there will be further clinical trials to explore whether it actually works to improve patients’ symptoms and prolong their lives. Hopefully, this is something we shall be looking at in the UK in the near future.

Oranges and lemons...Should patients diagnosed with MDS start taking vitamin C?

It is probably too early to say at this stage but it is certainly worth bearing in mind the many other health benefits of eating fruit, particularly citrus fruits such as oranges and lemons, as part of a well-balanced diet. Perhaps we should all be listening to the good advice of the bells of St Clement’s?

More than 50% Watch & Wait patients thought about MDS to a significant extent compared to those placed on active treatment or supportive care (see graph below)

Reaction to being placed on Watch & Wait differs amongst patients, some patients are able to cope well with it. However, some may suffer from anxiety or depression as a result of being placed on Watch & Wait. Anxiety or depression might affect some aspects of their lives; it might lead to difficulties relating to their work life thus resulting in temporary leave of work or reduction in working hours. This might have some financial consequences to the patient.

The feeling of being anxious or depressed is aggravated by the lack of information at the time of diagnosis. Based on our survey,
38% Watch & Wait patients did not receive any written information at the time of diagnosis
43% Watch & Wait patients received minimal verbal information at the time of diagnosis

These figures illustrate the importance of providing adequate information to patients. If patients are provided with information in a patient friendly and personalised manner at the earliest stage of diagnosis, this might reduce the worry and help them cope well with being placed on Watch & Wait. Likewise, it will reduce the time a patient spends in consultations with the clinical team, or even with a counsellor, thus freeing up time to see other patients.

When asked about the greatest challenge of having MDS, Watch & Wait patients said:

‘‘The Watch & Wait policy. Regular 6x weekly visits to the hospital for blood tests then lack of feedback on the results’’
‘‘The uncertainty over whether there will be progression of the disease’’
‘‘Watch & Wait is a bit like having a sharp sword hanging about your head. It seems to get lower every time you are heading for your next blood test’’

In comparison, patients on active treatment/supportive care said:

‘‘The side effects of Azacitidine’’
‘’The disruption to our lives. Unable to plan ahead as Chemo dates can change if my blood is not right. Having platelets every week is a real nuisance’’
‘’Waiting 8 hours for blood transfusion’’

Support for MDS patients and carers

There is a constant need to access information and advice for MDS patients and their carers. Access to these services provides them with knowledge as they face several challenges throughout their MDS journey. Access to a clinical nurse specialist, appropriate signposting to services and peer to peer support have been found to be useful for MDS patients.
We currently have local groups in several parts of the UK. If you would like to attend any of our meetings, please visit our page for further details or call us on 020 7733 7558.

Access to MDS Nurse or Haematology Clinical Nurse Specialist

Question: Do you have access to a specific MDS Nurse or Haematology CNS?

6% reported they had no access to a CNS but would like to see one 19% reported that they had never been offered this service
It is important that MDS patients have access to a Clinical Nurse Specialist (CNS) because they play a vital role in the management of patients concerns and support their health and wellbeing. We liaise with Clinical Nurse Specialists in several hospitals across the UK to provide assistance and to make it known that support and care is available for MDS patients.
According to the National Cancer Patient Experience Survey (NCPES), having a CNS is an important factor found to be positively associated with a better patience experience.1
To understand the importance of CNSs, we divided the patients who answered the question, ‘’Do you have access to a specific MDS Nurse or Haematology CNS?’’ into five categories based on their level of worry.

Question: How frequently did you worry about MDS in the last 4 weeks? (The traffic light colours of green, yellow/amber and red are used to encode positive, neither positive or negative and negative responses.)

40% of those who had no access to a CNS but wants to see one and 52% of those who had never been offered the service, worried about MDS to a significant extent. The lack of access to a named CNS aggravates the emotional impact of MDS and the findings of our survey illustrates this.

MDS patients are often confused at the time of diagnosis mainly due to the fact that they have never heard of disease; having a CNS as a main point of contact makes it easier for patients to ask questions pertaining their diagnosis and treatment thus reducing the emotional impact of MDS. CNSs can also recommend additional support services to patients should they need it. Likewise, having access means that CNSs can quickly recognise any developing issues that may require attention thus potentially avoiding any major problems.

As the ageing population continues to increase, so will the challenge of supporting patients with MDS. The availability of treatments and development of new ones means that patients will live longer with the condition thus illustrating the importance of CNS as patients will need continuous support over the years.

Information about MDS UK at the time of diagnosis

Question: At the time of diagnosis, did you receive information on MDS UK Patient Support If not, how did you hear about MDS UK Patient Support Group? 

More than half of the participants said they did not receive any information on MDS UK at the time of diagnosis.
Half of the participants who had not received information about MDS UK, learnt about MDS UK via the internet.

Due to lack of information on MDS at the time of diagnosis, patients and carers have to take active steps when trying to find out more information about MDS. This makes them vulnerable to coming across unreliable, inaccurate and out of date online information.

Recommendations

In the long term, providing information at the earliest stage of diagnosis to all patients can really save substantial time and concern to all involved (patients, families, clinical staff).
Practical Suggestion: Some haematologists take it a very helpful step further: after seeking express consent from the patients, they copy MDS UK Patient Support Group into the patient’s clinic letter requesting us to send information to the patients. This step takes only one minute during consultation and ensures no patient goes potentially unsupported.