Posture issues in CF

What is posture? Skeletal posture image

The term posture relates to the position of the body, and the relationship between each of its parts. Our bodies are in a posture every minute of every day, they can be static, such as when we are resting or sleeping, or dynamic, such as when we are walking or playing sport.

Every posture that we get into has a demand on the muscles, joints, nerves and connective tissue of the body and some postures can become painful due to putting stress onto some or all of these components.

Posture is affected by many factors such as your mood (ever noticed your shoulders slumping if you are feeling low?), environment, sex, general health and personal habits.

What is ideal posture?

Ideal postures allow the body to maintain a position that doesn't put excessive strain onto any one part of the skeleton or muscles. For example, when standing, your head should be in neutral with the shoulder blades lying flat against your back, your spine should maintain its natural curves and your knees should not be locked back straight (see figure 1). 

An easy way of assessing good posture in standing or sitting is to imagine a plumb line running down the body, as shown in figure 1 and 2.

Why do I need to think about my posture? Posture image 2

Thinking about posture is important for everybody, as it can prevent injuries and pain in day-to-day life.

Sitting in a good, well supported position during study or work time can help prevent back and neck strains which can cause pain. Making sure that your mattress and pillows are supportive and comfortable for you are essential, as a lot of time is spent in this posture.

The CF component

Research has shown that people with CF are at high risk of postural deviations and pain as well as being more likely to develop osteoporosis or osteopenia (reduced bone mineral density), which can lead to pain and postural adaptations.

Risk factors for the development of postural and bone complications have been identified and include reduced FEV1, low lean body mass, CF related diabetes, corticosteroid treatments, pancreatic insufficiency, physical inactivity, low vitamin D, chronic pulmonary infections, delayed puberty and the Delta F508 genotype.

Investigations are ongoing as to whether the CFTR defect, which causes CF and which has been identified in bone and muscle cells, is a primary cause of bone complications, especially low bone mineral density, or whether postural problems are a secondary complication of the disease process of CF.  It has been proposed that the altered cough and breathing patterns plus inflammatory processes, alongside decreased activity levels that occur with pulmonary infections, are the main culprits of postural complications. 

Repeated pulmonary infections can lead to decreased lung function and more difficulty breathing, the muscles of the trunk work to facilitate breathing while also supporting posture, and the more demand placed on them for breathing, the less ability they have to support your posture. This can leave the skeleton at risk of postural complications or deformities from excess forces acting upon it, for example coughing or prolonged slumped postures. 

Prolonged infections or periods of inactivity can lead to the soft tissues such as muscles, adapting to non-ideal resting postures. An example of this is when the chest wall muscles shorten from sitting slumped with rounded shoulders making it difficult to straighten up and sit tall. If this happens, it is very hard to be able to move your shoulders fully or take a deep breath.  The longterm consequences of this can include a decreased ability to perform airway clearance and spirometry, and less ability to exercise, which can then create a vicious cycle of being more at risk of chest infections and becoming more unwell.

What do you mean by postural complications?

Postural complications often start out with soft tissue changes, such as altered muscle lengths due to adaptations to a non-ideal posture, which can lead to decreased flexibility or decreased range of movement of a joint.

Typical body areas affected in CF are the shoulders, ribcage and upper back. Alterations in the way muscles work exerts a new direction of force or lack of support to the bones they attach to, pulling them in non-ideal directions, which can lead to skeletal deformities, such as increased thoracic curvature – called dowager's hump or thoracic kyphosis, which in turn can put the spine at risk of vertebral fractures.

Pain can occur throughout all of these changes as the body adapts to altered positions, and restrictions of movement. Reasons for discomfort include painful trigger points within shortened muscles, stress on weak points of the skeleton and the overstretching of body parts when in non-ideal postures. 

Who does this affect?

Occurrences of postural abnormalities are more likely as you get older, and as your disease progresses, especially with more chest infections and increased cough.

Currently, there are no studies reporting postural abnormalities in children under six years old. Numerous studies have noted the emergence of musculoskeletal abnormalities in children nearing puberty, particularly in relation to low bone mineral density.

As we look at the adult population, pain from postural adaptation, especially low back pain, is reported with increasing frequency, with some studies reporting an incidence as high as 94% in their patient group. In a non-CF adult population the incidence of low back pain within a person’s lifetime is estimated at 50-80%, suggesting that you are more likely to develop low back pain if you have CF.

With the pleasing prospect of life expectancy of a person with CF increasing, it is likely that we will see even more postural complications occurring, due to CF-related bone disease alongside the wear and tear of old age and a more active lifestyle. It is therefore important that every person with CF is aware of the possibility of postural complications, and knows what to do about them.

What can I do?

Low bone mineral density is linked to postural complications, so it is really important that you are compliant with your vitamin D and calcium supplement therapies as these will help you to build and maintain strong bones.

About 90% of adult bone mineral is acquired by late adolescence, with peak bone mass reached at 20-30 years of age so this is the key time to work on consolidating bone mass. Liaison with your CF dietician about optimising these supplements, alongside trying to maintain a healthy weight are essential in the prevention of bone disease and postural complications.

In a non-CF population weight-bearing exercise such as jumping, has been shown to maximise peak bone mass, strength and size, while also reducing bone loss. There has been evidence to show good effects of weight bearing exercise on bone in young adults, with some carry over into later adult life. A group of experts in the field of CF bone disease have produced a consensus document that recommends weight bearing exercise for people with CF as part of the management of bone disease.

The best way to tackle postural complications is to prevent them happening in the first place, always being aware of your posture and making sure you stretch out if you have been in a non-ideal posture are easy ways of incorporating posture work into everyday life.

Talking to your physiotherapist about your posture can be really helpful as they can suggest positions to optimise posture that can be utilised with nebuliser therapies or airway clearance techniques.  

We have included a posture review as part of your annual review, which allows us to highlight areas to be aware of, and we can suggest exercises that will help to correct non-ideal postures or stretch out tightened muscle groups. Often self-management of posture can be easily fitted in to daily life without increasing your treatment burden. If problems become more complex, such as low back pain due to decreased spinal mobility, then hands-on therapy can be effective in helping to ease discomfort. The need for these types of interventions will be assessed by your physiotherapist. 

Action plan for optimal posture

1. Get assessed

An easy self-assessment of your posture is to look at yourself in the mirror, either sitting or standing. Imagine the plumb line running down your body (figures 1 and 2), try to alter your position so it fits the ideal picture.

Talk to the physiotherapy team about your posture. They can then help you devise a posture management plan involving a range of interventions from being more aware of your position throughout the day to specific exercises to focus on problem areas.

2. Optimise your diet

Liaising with your CF dietitian is essential to make sure that your diet is optimal for bone health. Ensuring that you have good levels of vitamin D and calcium in your diet or taking your supplements if they are prescribed for you, as well as keeping a healthy weight are all really important factors in having strong bones to help in preventing postural problems.

3. Participate in weight-bearing activities

Whatever age you are, weight-bearing exercise is thought to be beneficial to bone health, which in turn will help prevent postural complications. The best sort of weight-bearing exercises are either high-impact loading exercises such as jumping or odd impact exercises such as ball games or gymnastics where you have lots of quick changes of direction.

To work on all areas of your body you will need to change the area that is weight bearing. For example, holding your body weight with your arms as you would in yoga or gymnastics is important for your upper body, while weight bearing through your legs, such as in jumping, is important for your lower body. Remember these activities also have positive benefits on your chest and general health so you can be optimising several things at once!

4. Maintain flexibility

The body is able to maintain ideal postures easily when its muscles are able to contract in the most optimal way. Working to keep muscles as flexible as possible will help this, as well as preventing pain from uneven pulls on the skeleton from shortened muscles. Making sure you stretch after exercise will prevent shortening of muscle groups, while yoga and Tai Chi have been shown to improve posture, strength and flexibility while having a positive effect on mood and a possible beneficial effect on bone mineral density.

5. Prevent injuries

If you do have low bone mineral density or osteoporosis (diagnosed by your medical team after dexa scanning), it is important to maintain exercise while being careful not to put yourself at risk from injuries.

Simple principles to follow are avoiding exercises or activities that flex, bend, or twist the spine and limiting high-impact exercise to lower the risk of breaking a bone. 

It is recommended that you consult an exercise specialist or physiotherapist for advice on proper progression of activity, how to stretch and strengthen muscles safely, as well as how to correct poor posture habits.

References

ACPCF Standards of Care and Good Clinical Practice for the Physiotherapy Management of Cystic Fibrosis, Cystic Fibrosis Trust, 2nd Edition, June 2011.
ACPRC Guidelines for the Physiotherapy Management of the adult, medical, spontaneously breathing patient. Thorax 2009; 64(Suppl I)

Musculoskeletal and Neuromuscular Interventions: A Physical Approach To Cystic Fibrosis, M. Massery. Journal of the Royal Society of Medicine 2005; 98 (suppl.45): 55-66.

Posture and Cystic Fibrosis, R. Tattersall and M. Walshaw. Journal of the Royal Society of Medicine 2003; 66 (suppl. 43):18-22

Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood With Cystic Fibrosis, R. Aris et al. Annals of Internal Medicine 1998; 128 (3): 186-193

Immediate Effects of Musculoskeletal Physiotherapy and Massage on Pain and Work of Breathing in Adults With Cystic Fibrosis, A. Lee et al. The Journal of Cystic Fibrosis January 2009; vol 8 (1): 1569-1993

Posture in adult inpatients with cystic fibrosis, Sandsund C.A., Robinson V., Banya W., Bilton D., Hodson M.E., Pryor J.A. Journal of Cystic Fibrosis, 2010, vol./is. 9/(S72), 1569-1993

Cystic fibrosis core strengthening and respiratory exercise program (CSREP). Christiansen J., Thompson L., McNamara J., Johnson M., Fenlon K. Pediatric Pulmonology, 2010, vol./is. 45/(387), 8755-6863  


Cystic fibrosis centre: 

Fulham wing, Royal Brompton Hospital, London, SW3 6NP

Telephone: 020 7351 8065

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