Ageing with spina bifida

1000 Voices - what did you tell us?

Shine surveyed its adult members with spina bifida to find out how their condition affects their health and lifestyle, and how this is changing with their passing years. Little is currently known about how spina bifida affects the ageing process, and with so many of our members aged 40 or over, we think it's important to get answers to some of those questions.

Of Shine’s 3,427 members with spina bifida aged 25 or over, an impressive 1082 completed the survey, (online or the paper version posted out in April), despite the questionnaire being very long (and pretty nosy in places!).

Driving

So often a lifeline and passport to freedom for many people with restricted mobility, especially in rural areas, we wanted to know how many people drove, where, and if they stopped driving, why.

67% said they have held a full driving licence, and 81% of those were still driving (about two thirds drove wherever they wanted, and a third drove in their local areas or routes they know well). Of the people who had stopped driving, the most common ages were 36-40, and 21-25.

People had stopped driving for many different reasons. Some people aged 25-30 had found it too expensive, but other groups gave mainly medical/health reasons, such as concentration issues, anxiety/depression or sleep apnoea for stopping driving. Some of these conditions might be improved with information and support, which might help keep some people on the road with the freedom and independence that can bring.

Work

As the survey went to members of all ages, many of our members were of retirement age. When we looked at working age people (25-64), 59% said they’re not working at the moment. This is lower than the national figures, which has 76% of working age adults in some paid work. 13% of working-age members were in paid work, and another 16% in part time paid work.

The proportion of people not working generally increased with age from 52% at 25-30 to 70%+ when over 56. Over half of members said their work situation had changed over time

The most common changes were:

from working to not working;
reduction in working hours;
from paid to unpaid work;
and changes staying within paid work.

 The most common age range for changes in work to occur was  36-40 (22%), 46-50 (16%), and 26-30 (14%). The median age for work changes to occur was 40. 

Of the physical issues that led to changes in work, bladder/bowels, pain and anxiety/depression were the most common. Other changes included redundancy and retirement, including medical retirement. Many members generously shared their experiences of tribunals, roles being changed, and being too worn out to work any longer.

This is clearly an area for Shine to look more closely into.

Living arrangements

The most common living arrangement was living with a partner, which was 38% of people. Two thirds of 25-30 year olds lived with parents, reducing to one third of 31-35 year olds. The reasons given for living with parents were:

happy there (58%)
high care needs(42%)
financial reasons (31%)
anxiety about moving out (28%)
and lack of accessible housing (21%)

The proportion of people living alone increased from 6% of 25-30 year olds to 48% of 66-70 year olds. We looked at ways living alone might influence health, for example depression or pressure sores, and found that people living alone were no more or less likely to have health issues than those living with others.

Mobility

We collected a great deal of information about the ways people get around. 17% of people walk everywhere unaided, but many people told us their mobility had changed over the years. Around 40 years old seemed to be the time of most change and was the commonest age for starting to use a power chair. Two thirds of people had used a wheelchair for more than 20 years and one third of people who used sticks or crutches had done so for over 15 years. Wheelchair use and use of sticks can result in damage to the hands, neck and shoulders, and we looked at pain and use of the hands and shoulders, to see how they relate to equipment for getting around. People who walked unaided were more like to have no issues with their upper body, all other groups reported more pain and loss of use of their hands. Could starting to use a power chair earlier prevent some pain and loss of independence? We need to look into this more closely.

Many people commented on how the change in mobility came at the same time as other health changes, like pain and weight gain, and we felt the frustration many people experienced as their lives changed, often very drastically.

Leg movement had also reduced for many people, most commonly between 25 and 40, with a median age of 35. Members have often told us things began to change at around 35. Is wear and tear on tethered spine behind some of these changes?

Balance difficulties can make going out very hard and create a lot of anxiety. The numbers of people telling us their balance had got worse over time increased from 32% aged 25-30 to between 80% and 94% in people 61 and over. Women were more likely than men to report increasing difficulties with balance (74% compared with 42%). 

We asked members about problems in their neck, shoulders and hands, such as difficulty moving them, or pain. Only 31% said they had no problems, with 45% having two or more problems. The most common age for upper body problems to start was 36-40. Many people said it made things harder and more painful, but it didn’t stop them doing anything. 30% of people said their hand movements were affected, which is concerning, since this could affect transfers and self-care, such as catheterisation, dressing or cooking. We couldn’t see any relationship in our members’ responses between upper limb issues and the length of time using manual wheelchairs or crutches, although we know that damage can occur. People in pain were more likely to exercise their shoulders after using their wheelchair or crutches.

Bone health

Bone problems begin at a young age for our members. 52% of people aged 25-30 reported at least one bone or joint problem, rising to 75% in people over 51, and 86% in those over 71. Surprisingly, 9% of our members aged 25-30 said they had osteoarthritis, rising steadily to 59% of those over 71.

Osteoporosis is strongly associated with age. People, especially women after the menopause, experience loss of bone density and strength. Reduced weight bearing (wheelchair use), reduced kidney function, and some medication for epilepsy increase the risk for osteoporosis and it‘s thought around 33% of people with spina bifida may develop osteoporosis, higher than the general population. In the general population in the UK, around 2% of 50 year old women and 21% of women over 50 are thought to have osteoporosis., while around 7% of men over 50 are thought to have it. 

In our survey, 7% of men aged under 50 and 10% of men aged 51 or over, 10% of women under 50 and 22% of women aged 51 or over said they had osteoporosis. Our results show the risk of osteoporosis, especially in men and younger women is far higher for people with spina bifida, but that it still may be under-diagnosed in the UK. Bone density screening is usually done for women past the menopause. Our members, men and women, may need extra screening for osteoporosis, throughout their adult lives, and beginning in their twenties.

Skin and tissue health

We asked our members about cellulitis, pressure sores and lymphoedema, and their experiences as they got older. These conditions are often linked, for example lymphoedema can make pressure sores more likely. A third of our members had had cellulitis, typically starting at 21-35 years old. Similarly,34% of respondents had experienced lymphoedema, the proportion increasing with age from 20% in 25-30 year olds to 43% in 56-60 year olds, beginning at a median age of 35. This is much higher and younger than the general population. Lymphoedema can affect mobility, independence and self-esteem, as it can become hard to move heavy legs, and people told us it restricted their choices of clothing and shoes. The good news is that 45% of people had had compression treatment, where carefully fitted stockings or wraps squeeze the excess fluid back into the bloodstream. It is very effective, and those members with lymphoedema not having treatment might want to request an assessment from their GP.

Pressures sores are more likely for people with spina bifida; reduced skin sensation, reduced mobility, bladder/bowel issues and over, or under-weight, can all make them more likely. They can lead to severe ill-health, even amputation, and once there, can take a lengthy period of onerous treatment to heal. Over half of our members had had at least one pressure sore, with more men than women, and 57% of people with sores had been in hospital for treatment, typically for 2-3 weeks but sometimes for months.

While some people said they’d had many sores since childhood, the most common ages were between 11 and 30. During the teen years, rapid growth can lead to changes in skin sensation and circulation if the cord is tethered. It’s also the age where people tend to want more independence and privacy from their parents. Since the most common areas for sores were the buttocks and feet, areas difficult to see, does watching for skin reddening and marking need to be built into the daily routine for young people from 11 up?

Looking at all the responses for skin and tissue health, only 22% had had no issues, while 48% had two or more, of lymphoedema, cellulitis and pressures sores.

Chiari II and tethered cord

Chiari II is a developmental condition of the cerebellum, part of the back of the brain. It is associated with myelomeningocoele, the commonest form of open spina bifida. Most people with myelomeningocoele have Chiari II, but until MRIs became more readily available, many adults with spina bifida may not be aware of it. Occasionally it causes issues in infancy and childhood, such as swallowing problems and low muscle tone, but may never cause physical issues. However, some adults develop symptoms in adulthood, especially middle age, and we wanted to know more about this. The numbers of adults telling us they have Chiari II fell from 25% of 30-35 year olds to 10% of 56-60 year olds, possibly through differing awareness, but possibly more people finding out as adults they have closed forms of spina bifida, which is not associated with Chiari. 58% of people who knew they had Chiari had symptoms, most often starting in their 30s, although the median age was 26. A quarter had had surgery (decompression, shunting, detethering), most often in their 20s.

Tethered cord, where the spinal cord becomes ‘stuck’ in the spinal column, and unable to ride up in the spine during growth, can cause symptoms during childhood such as pins and needles ‘growing pains’, cramp, and changes to the shape of the feet. Sometimes the tethered cord causes no symptoms until adulthood, when the spinal cord begins to deteriorate as a result of being tethered for many years. 28% of people said they’d been told they have tethered cord. After childhood diagnosis, the most common time to find out was between 36 and 45. Our members have told us for many years that things had changed at this time, with reducing mobility, increasing pain, and bladder/bowel involvement. Some people had had several detethering surgeries, but, again, after childhood, the most common ages for detethering were 46-50, and 21-25.

With the cord becoming tethered during the teen years, Shine needs to know whether detethering surgery later in life has helped with movement and quality of life, we may come back to our members for more information.

Kidneys and blood pressure

People with all forms of spina bifida are likely to have neuropathic bladder issues. The nerves to the bladder and sphincters can interfere with filling, storing and emptying of the bladder. Urine retained in the bladder can become infected, and tight bladder muscles can drive urine up to the kidneys. This can damage the bladder and kidneys, causing scarring, and infections can lead to stones. The stones can then lead to further infections, setting up a cycle.

A quarter of members told us they’d had renal or bladder stones, almost double the lifetime rate for the wider population, and 37% said they had reduced kidney function.

Blood pressure can be influenced by kidney function, but high blood pressure (hypertension) can also damage the heart and kidneys. Other factors, such as being overweight or being physically inactive can also raise blood pressure, and these are common in adults with spina bifida. In our members, the proportion of people with high blood pressure rose with age until the over 65s, then dropped a little. 6% of people aged 25-35 had high blood pressure, this rose to 51% of people aged 60-65. 43% of men had high blood pressure, compared with 31% of men and 26% of women in the wider population. The median age for getting diagnosed with high blood pressure for the first time was 40, which is young.

1 in 20 of our members didn’t know if they had high blood pressure and hadn’t been checked. High blood pressure is very dangerous; left untreated it can lead to strokes and heart failure, as well as kidney damage.

We strongly urge all adults to have at least yearly checks for their blood pressure

Bowels

Good bowel management is a key factor in quality of life for adults with spina bifida. Neuropathic bowel can result in severe constipation and leakage. Constipation with overflow can cause enormous management problems, with sudden and unpredictable episodes of explosive faecal fluid, difficult to contain. Many members talk to us about frequent ‘tummy upsets’, or that certain foods ‘upset them’, especially vegetables. However, on questioning, there is often a pattern of mostly severe constipation, with intermittent explosions. Overflow is caused by constipation, and only resolving the constipation will prevent the explosions. Diarrhoea medication slows the bowel, leading to more constipation, (although it temporarily halts the explosions for a few hours) making the condition worse.

Intermittent explosions were experienced by people of all ages, but more for people aged 56-60 (25%) if you include all methods of management, but more so for those using medication alone to manage. It may be that this can be improved for you, so ask us if you’re bothered by intermittent bowel explosions.

The good news is, a third of members have full bowel continence, and nearly as many only soil once a month or less. But around half of people aged 56-60 soil once per week or more. It can be harder for women to maintain bowel continence after the menopause, so getting bowel management as good as it can be, as early as possible, can support general health, protect the buttocks from pressure/moisture sores and help keep people in employment (Some people had left work because of their bowels deteriorating).

Mental Health

There are many reasons why people with spina bifida might experience poor mental health. Many people report pain, reducing mobility, difficulties with bladder and bowel, long periods on bed rest for pressure sores. Many of our members are not working and some are socially isolated, owing to society’s attitudes and the built environment, with inadequate or restrictive care which denies autonomy, and more recently, changes to the benefits’ system.

The cognitive effects of spina bifida can result in anxiety (decision making, forward planning, managing change, processing of information) and depression. Although 26% of the wider population experience mental ill health at least once in their lifetime, only 30% of our members said they had no problem with their mental health.

Our survey results showed that 53% of respondents experienced depression, and 56% anxiety.

87% of people with anxiety felt their mental health had stayed the same or deteriorated.   Only 12% of respondents reported that their mental health has improved over time. Anxiety and depression were strongly associated with pain, Chiari, sleep apnoea symptoms. Numerous comments talked about deteriorating health, independence and mobility.

The full list of health associations is below. Anxiety can potentially be improved by use of techniques such as mindfulness, or therapy such as CBT which we’ll look further into.

Having depression or anxiety was associated with many other issues, respondents with these mental health problems were: 

More likely to be unemployed (63% for anxiety and 63% for depression) compared with those with no mental health problems (56%). They were also less likely to be in full-time work: 11% for anxiety and 11% for depression, compared with 17% of people without mental health issues.  
More likely to report their mobility changing over time: depression (83%), anxiety (80%), no mental health problems (67%). 
More likely to have reported the movement in their legs reducing over time: depression (62%), anxiety (59%), no mental health problems (47%). 
More likely to have experienced deteriorating balance: depression (75%), anxiety (74%), no mental health problems (61%). 
More likely to experience shoulder pain and neck pain: depression (61% - shoulders, 57% - neck), anxiety (60% - shoulders, 56% - neck), no mental health problems (38% - shoulders, 34% - neck) 
More likely to have bone/joint disorders: depression (74%), anxiety (71%), no mental health problems (58%). 
More likely to have Chiari II: depression (21%), anxiety (22%), no mental health problems (10%). 
More likely to have tethered cord: depression (32%), anxiety (32%), no mental health problems (24%). 
More likely to experience some degree of soiling: depression (72%), anxiety (74%), no mental health problems (65%). 
More likely to have one or more symptoms of sleep apnoea: 
Tired every day: depression (65%), anxiety (63%), no mental health problems (26%). 
Snore loudly: depression (41%), anxiety (38%), no mental health problems (26%). 
Fall asleep during the day: depression (36%), anxiety (36%), no mental health problems (20%). 
More likely to experience pain 6-7 days a week: depression (57%), anxiety (53%), no mental health problems (38%), and less likely to experience pain rarely: depression (19%), anxiety (18%), no mental health problems (36%), 
More likely to report worsening pain over time: depression (68%), anxiety (66%), no mental health problems 52%). 
Less likely to feel their spina bifida was very well understood in hospital: depression (19%), anxiety (18%), no mental health problems 33%).

Obstructive sleep apnoea

Obstructive sleep apnoea is a sleep disorder where breathing is interrupted by a narrowing of the airway caused by relaxation of the throat muscles and obstruction of the windpipe. Symptoms of sleep apnoea when asleep include: loud snoring, stopping breathing or choking, and tossing and turning during the night. Daytime symptoms of sleep apnoea include: falling asleep during the day, not feeling refreshed after a night’s sleep and instead feeling groggy and tired, headaches are common, as are difficulties with memory and concentration. 

People with Chiari II are at increased risk of sleep apnoea, as are overweight people with excess weight in the neck area. It affects around 3% of UK adults. Our survey showed, a high proportion of members had been diagnosed with sleep apnoea (10%), and that more men (14%) than women (8%) had been diagnosed.

Of the members diagnosed, 67% had been treated, and three quarters of them thought the treatment worked well.

The treatment can take a lot of getting used to, and some people couldn’t tolerate it. But, a further 51% had two or more symptoms, such as feeling tired all the time, and falling asleep in the day without knowing why.

Apart from the poor quality of life OSA brings, it is damaging to health, and can lead to heart damage and other organ failure. It is easily diagnosed through sleep studies, and treatable for many people.

Pain

Pain is generally a mechanism to alert the brain to damage being done to the body, so evasive action can be taken. From an evolutionary point of view, it was more important to know whether you were injured or not at times of danger, so there is a well-established link between pain and anxiety.

Anxiety increases the experience of pain. Pain can also increase anxiety - (for example-what is the pain? Is it something serious?) creating a vicious cycle. Pain interferes with sleep, and everything is harder to deal with when you’re sleep deprived. Long-term pain can change the way the brain handles pain. Chronic pain can exist without any physical problems, or well after an injury is healed.

Damaged nerve pathways can result in neuropathic pain and distort the pain messages to the brain. With so many reasons why people with spina bifida might be in pain (neuropathic pain, bone infections, head pain from shunt issues, wear and tear on joints from mobility equipment, differing leg length, curvature of the spine, atypical gait) we wanted to know how this changes as you get older, and how it affects your lives.

Only 25% of members told us they rarely experience pain. The proportion of people saying they had pain 6-7 days a week, generally increased with age, from 40-41% in members aged 25-35, up to 66% in people aged 61-65. The numbers of people saying they rarely had pain also went down with increasing age, from 30-35% in those between 25 and 45 years old to 5-17% in the people aged 61 and over. Members shared the impact of living in pain on their lives, the effect on their mental health and the exhausting reality of life lived in pain.

Menopause

Shine was not aware of any studies into the age of menopause for women with spina bifida. The ‘received wisdom’ was, the earlier menstruation starts, the earlier menopause would occur. I

n the UK, the average age for women having their last period is 51, in our members the average was 49, which is well within the typical range. Since early and precocious puberty are associated with open spina bifida and hydrocephalus, some women will have periods longer than average, which could have implications for those cancers that are influenced by oestrogen, such as breast or ovarian cancer. Women with spina bifida should attend their routine screening tests, particularly their mammograms.

Later menopause might help protect against osteoporosis, since this tends to worsen once oestrogen levels drop. HRT can be prescribed for menopause symptoms and bone health, but only 28% of women members who had been through the menopause were prescribed HRT

Prostate

The prostate is a gland which surrounds the male urethra at the base of the bladder, which secretes semen.

Throughout life, it enlarges; from the age of 45-50 it can begin to obstruct the bladder neck and can lead to retention of urine and urinary tract infections. In men with spina bifida, it can begin to make self-catheterisation more difficult.

However, in addition to this benign enlargement (Benign Prostatic Enlargement/Hypertrophy or BPE/BPH) 1 in 8 men will develop prostate cancer, and the risk is higher if you have a family history or are black.

The prostate is well hidden, and you can’t examine it yourself, so talk to your GP if you are over 50 (over 45 if you are black or have a family history of prostate cancer) about prostate checks.

In the UK, 40% of men aged 50 will have enlarged prostate, rising to 90% of 80+year olds. In our survey, only 8% of men over 50 told us they had an enlarged prostate.

As there’s no medical reason why men with spina bifida wouldn’t have similar rates of enlarged prostate, is this low rate owing to lack of awareness? We urge all men over 50 to ask the GP for regular checks of their prostate size and shape, and to do blood test for prostate cancer (PSA)

Healthcare

With the complexity of spina bifida, and the many ways it can affect physical and mental health, we wanted to know what healthcare you were able to access, and whether proactive checks, rather than seeking help when you’re ill, was common.

Only 15% of members were able to access care from a multidisciplinary team, and a quarter weren’t under the care of any specialists. Seeing your doctor when ill was more common than having proactive checks.

Half of our members were under the care of a Urologist, which is good to know, given the higher risk of people with spina bifida for bladder cancer.

People who weren’t under the care of specialists were more likely to have had a pressure sore, and more likely to be in pain most days.

More than half of members said they had had an emergency admission to hospital in the last 5 years. 14% had had several admissions.

While most admissions were for less than a week, some were for many months, and the average was 20 days. Skin/tissue problems were the commonest reasons for admission, followed by urosepsis.

Once in hospital, only 24% felt their spina bifida was always well understood, 43% found it difficult or impossible to get specialist equipment, 54% found it difficult or impossible to stick to their bowel routine, and a shocking 23% had acquired a pressure sore while in hospital

Other findings...

The comments people shared with us were broadly:

Lack of understanding, interest, and specialist knowledge of healthcare professionals, particularly GPs.
Receiving reactive rather than proactive care to the detriment of health.
Delays and difficulties accessing treatment, and specialist care, physiotherapy especially.
Having to pay for private consultations and care, extra expense.
Low standard of care.
Lack of support and advice.
Lack of understanding and support for health needs associated with ageing

There is clearly much that could be done, to make specialist care by multidisciplinary teams the rule rather than the exception, to support members to communicate their needs and educate health professionals caring for them.

There’s a lot to do, but now we have some evidence to take forward. This will form the basis of our work for years to come, guiding members and professionals alike towards the middle years, and beyond, with spina bifida.

Thank you to all who added their voices, and thank you to the volunteers for the data inputting. We are very grateful.