illustration of a questionnaire or survey with lines of text and tick boxes on a lap top screenUpper Limb Study

Why did we do this study?

As the majority of our beneficiaries are upper limb affected, we wanted to study what the long-term effects were of having an upper limb difference and to compare the effects of those who have had surgery versus those who have not.

Background of the study

In 1977, a surgeon called D W Lamb published a paper on the treatment of 117 people affected by radial dysplasia - where one of the wrist bones doesn’t form properly - in thalidomide patients at the Princess Margaret Rose Hospital, Edinburgh.

In 1997, this study was followed up by recruiting some of these patients to return for formal assessment. 17 patients were assessed in person and more by case notes and X-rays. Their symptoms and psychosocial status were looked at.

It has been 20 years since the last study. Our beneficiaries are now in their late fifties and early sixties. We wanted to explore what long-term issues they have, both clinically and psychosocially.

Who did we do the study with?

The study lead was a congenital hand surgeon called Mr Wee Leon Lam. Mr Lam was a medical student at the time of the 1997 study and assisted with it and thus had a real interest in those with the embryopathy.

Mr Lam had a team working with him on this study that included Mr Geoffrey Hooper, a retired surgeon who has seen and treated beneficiaries, Mr Paul Stirling, a senior surgical registrar, and Magdalena Markiewicz, one of Mr Lam’s medical students. The study was done in conjunction with Mid Lothian NHS trust, where Mr Lam works as a surgeon.

How did we do the study?

An online questionnaire was developed by Mr Lam and his team. This questionnaire was sent to every beneficiary with upper limb damage. The questionnaire incorporated standardised questionnaires used in other studies that asked about various aspects of how living with an upper limb disability affects you.

These questionnaires included:

  • Quick DASH score. This questionnaire asks about your symptoms as well as your ability to perform certain activities, for example, doing household chores. It is scored out of 100, with higher scores reflecting increasing difficulty with activities of daily living and symptoms.
  • EQ-5D-5L questionnaire. The scale measures quality of life on 5 aspects of your life including mobility, self-care, usual activities, pain/discomfort, and anxiety/depression. It is scored from 0-1 with 1 being “perfect health” and the closer to 0 means poorer health.
  • Work and Social Adjustment Scale (WSAS) which assesses the impact of disability on your work and social life.
  • Derriford Appearance Scale (DAS-24) which looks at how beneficiaries perceive their appearance and levels of distress caused by their appearance.
  • Neuropathic Pain Score (NPS) which looks at neuropathic pain. Neuropathic pain is caused by damage or injury to the nerves that transfer information between the brain and spinal cord from the skin, muscles and other parts of the body. The pain is usually described as a burning sensation and affected areas are often sensitive to the touch. Symptoms of neuropathic pain may also include excruciating pain, pins and needles, difficulty correctly sensing temperatures and numbness.

Additional questions were asked regarding how easy it was for beneficiaries to access health care, how supported they felt and whether they felt health professionals had a good understanding of their health issues.

How many beneficiaries responded?

We had 127 responses from beneficiaries, of which 120 had differences to both arms. 17 beneficiaries who responded had a complete absence of an arm on one side or both.

Just over half of beneficiaries who responded had had some form of surgical treatment to one or both sides.

What we found

The average (mean) QuickDash score was 54. Remember higher scores mean increasing difficulties with different aspects of your life such as daily living and how this  affects you. This compared with just a range of scores of 11-20 in people of similar age with no thalidomide exposure.

In a similar study in Sweden (Jahani et al 2014), thalidomide affected individuals reported lower QuickDash scores of an average of 20.5.  At the time of the study, the individuals concerned would have been around 46 years of age. This supports the view that there is significant decline with age in performing in all activities of daily living, and the effect this has on living different aspects of your life.

The EQ-5D-5L which looked at quality of life, showed that beneficiaries had an average (mean) score of 0.55 compared to 0.89  in the general population. The majority of beneficiaries (70%) reported a deterioration in their quality of life. Again, there was a poorer score for our beneficiaries than in the thalidomide affected individuals in the Swedish study. Again, this is more evidence that the difference is most probably due to the Swedish study being 8 years ago and we know the deterioration that some of you have suffered from a health and wellbeing perspective.

Higher quality of life scores corresponded with higher scores associated with ease of doing activities of daily living and with improved symptoms. Therefore the more able you are to do daily tasks means the more satisfied you are with your life.

The average WSAS score was 15. Scores of between 10-20 demonstrates our beneficiaries have significant impairment in carrying our all aspects of their lives from socialising, to working, to jobs around the house.

For those that had had surgery, there was no significant difference in either neuropathic pain (NPS score), quality of life (EQ-5D-5L) or in the questionnaire that looked at symptoms and also activities of daily living (QuickDash).

Over a quarter of you that responded to the survey had symptoms suggesting neuropathic pain.

Almost a quarter of you reported difficulties accessing healthcare and almost half of you (47%) felt that there was a lack of understanding from health professionals about thalidomide and the complex health situations of our beneficiaries.

What do these results mean for you?

If you’re having issues accessing your GP or getting a referral to see a specialist, it’s important to talk to the Trust to see how we can help.

Almost half of you in the survey felt that health professionals didn’t understand your upper limb issues.  That’s why it’s important that your GP has a copy of the GP resource pack, and you can also take this with you to any health appointments.

You can download the GP resource pack from our website

Get in touch with the Trust if you are having difficulties conveying your problems to a particular health provider and we can help by talking to them or writing to them.

 

The results indicated the increasing difficulty you had with activities of daily living and how this is worsening with time. Please visit our section on the website looking at aids and gadgets to help.

 

A quarter of you had features suggesting neuropathic pain. Visit the Brain and Spine website for more information about neuropathic pain.

 

If this sounds like you, then there are some treatment options and ways to manage it; get in touch with one of the Medical Advisers at the Trust on 01480 474074 if you want to find out more.

What do these results mean for the Trust?

This study provides further evidence for the issues facing those beneficiaries who are upper limb affected and highlight the need for ongoing specialist care and support. It provides further evidence of the significant impact on ageing on those with an upper limb disability and how that has caused a sudden decline in both function and quality of life.

The Trust can use the findings to:

  • Provide information to the medical community and general public about the problems experienced by those affected by thalidomide embryopathy and who have an upper limb difference
  • Provide evidence that can help strengthen letters written by the Trust for beneficiaries who are upper limb affected, to help make a case for referral to appropriate specialists
  • Provide information to help claims for disability, other benefits and services in the future
  • Provide further evidence to support the Trust’s private referral scheme
  • Provide up-to-date evidence of the issues that face our beneficiaries and may be able to assist in future talks and negotiations with health departments, other bodies and Diageo

What are the next steps for the results?

The study results have now been published. This will help to raise awareness of both thalidomide embryopathy and those with an upper limb difference.

The results have been already been discussed as part of a thalidomide symposium at the International Federation of Societies for Surgery of the Hand and International Federation of Societies for Hand Therapy in London 2022. This was an important, international hand conference and was attended by members of the Trust clinical network including Professor Giddins, Mr Elliot Sorene and Mr Alastair Hunter.

 

You can read the full publication on the National Library of Medicine's website