Pregnancy, birth, feeding and hypermobile Ehlers-Danlos syndrome / hypermobility spectrum disorders

Rachel Fitz-Desorgher, Midwife, Hypno-Lamaze Teacher, Infant Feeding Consultant and Parenting Consultant

Please note: The following text cannot and should not replace advice from the patient's healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.

Introduction

Pregnancy and parenthood brings both joy and challenges for all women. For the woman with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorder (HSD), the increased laxity of tissues can present increased and additional issues for which careful planning and management can help. As well as maintaining your usual lifestyle adjustments, ensure that you see your GP, midwife and any other therapists (physiotherapist, occupational therapist, etc) regularly to ensure your progress and health is properly monitored. Consider providing your caregivers with a good birth plan which details any special requests and recommendations to protect your joints and skin.

 

Minor disorders of pregnancy

Varicosities

These are swellings of veins and are more common in pregnancy due to the increase in the hormone progesterone, which softens the smooth muscle of the blood vessels. They can occur in the legs (varicose veins), anus (haemorrhoids or piles) and the vulva/vaginal area (vulval varicosities). Simple self-help strategies can help reduce their occurrence and severity. 

 

Varicose veins

Try to maintain your usual exercise regime. Gentle walking and water exercise (swimming, hydrotherapy) can be particularly helpful. When sitting, try not to cross your ankles, and put your feet up and circle them to improve circulation. Support tights can help ease aching veins.

 

Haemorrhoids

Both internal and external haemorrhoids (piles) can be eased by avoiding constipation. Maintaining a healthy diet with plenty of fibre (fruit, vegetables, grains) and fluids will aid regularity of bowels, as will gentle exercise. Regular pelvic floor exercises can help improve circulation, your midwife or physio can teach you how to do these if you are unsure. Iron tablets can increase the risk of constipation so talk to your GP about alternatives if you are anaemic. There are a number of cooling gel products on the market and these may be worth investing in as they can also soothe the perineum after birth. Finally, your GP will be able to prescribe you effective creams and suppositories to relieve haemorrhoids if they are severe.

 

Vulval varicosities

These are the most uncommon of the varicosities but can be very painful. Pelvic floor exercises will improve circulation and gel pads can relieve pain and swelling. Standing for long periods can make them worse and resting off your perineum (try lying on your side on the sofa) can ease the fullness and aching. Ensure that your midwife is aware of your vulval varicosities as they can occasionally rupture during birth (although there is little that can be done to avoid this, forewarned is forearmed!)

 

Heartburn

This is a burning, acidic feeling in the oesophagus (gullet) caused by stomach acid leaking back up. Spicy foods, caffeine, alcohol, citrus fruit and (despite what friends may tell you) milk can all make symptoms more likely; whilst eating small regular meals, drinking water (to dilute the acid), avoiding late evening meals and lying on your left side at rest can all help improve symptoms. If symptoms persist, see your GP for a prescription for safe antacids.

 

Oedema

A common symptom of pregnancy, this is caused by fluid leaking into the body tissues and leads to swelling of the feet, lower legs, hands and, less commonly, other parts of the body such as the face, vulva and tummy. Keep legs higher than your hips at rest and avoid crossing your ankles. Consider wearing support tights and circle your feet regularly to improve circulation which, in turn, clears away tissue fluid. Hand oedema can lead to carpal tunnel syndrome which causes tingling and numbness in the fingers. Ask your physio for splints and simple exercises to reduce discomfort and swelling. If your oedema is severe it may be a sign of pre-eclampsia. Keep your regular appointments with your midwife so that your blood pressure and other health signs can be closely monitored.

 

Nausea & vomiting

A common feature in pregnancy and not easy to relieve. Most suggested treatments have not been shown in studies to be useful. However it appears that low blood sugar may be implicated, hence it often being worst in the mornings. Eating little and often, keeping blood sugar stable with a sensible mix of both simple and complex carbohydrates and avoiding any foods that trigger the nausea is the best strategy for reducing it until it naturally passes at the 16-week mark. Occasionally symptoms persist throughout pregnancy – eating little and often, resting well at bad times of the day and avoiding triggers will continue to reduce the worst of the nausea and vomiting. Your GP and midwife will monitor and support you and, if your symptoms are very severe, you will be offered medicines to help.

 

Headaches

Hormonal changes and an increased metabolism can lead to an increase in the occurrence of headaches. Increasing fluids and taking simple pain remedies such as paracetamol can help greatly. Sometimes headaches are caused by nasal congestion which is increased in pregnancy. Try steam inhalations and sniffing oils such as eucalyptus in common remedies (Vicks and Olbas Oil). It is essential that, if your headache is accompanied by nausea, flashing lights or is not relieved within an hour of taking paracetamol, you seek urgent medical attention as headaches can be an indicator of pre-eclampsia or dangerously raised blood pressure.

 

Tinnitus

Many people with hEDS/HSD suffer from tinnitus as a result of the instability of the bones in the middle ear. Extra pregnancy progesterone may make your tinnitus worse. Try using a white noise generator at night – there are many smartphone apps for this, and it is also possible to buy specialist pillows which you use together with your phone to help give more restful sleep. If you find that your symptoms are severe, ask for a referral to an ENT specialist who may be able to offer more help and support.

 

Sleep

Many people with hEDS/HSD complain of poor sleep and of their lack of deep sleep. Sleep can also be affected by pain, tinnitus, palpitations and poor core temperature control. Taking simple painkillers such as paracetamol before bedtime, using light bedding and a cool fan, and keeping as regular a bedtime as possible may help to protect your sleep.

 

Musculoskeletal problems

It is not uncommon for people with hEDS/HSD to say that their pain symptoms started in their first pregnancy. It is possible that the increased laxity of pregnancy does not settle properly postnatally.

In any event, women with hEDS/HSD need to be particularly careful to protect their joints throughout pregnancy and during the postnatal period. Regular check-ups with a specialist physiotherapist, carefully monitored use of painkillers, and timely appointments with other specialists such as podiatrists and occupational therapists can ensure that symptoms are reduced and managed.

Also important is avoiding excessive weight gain as extra weight puts more stress on our joints, and those with hEDS/HSD are more vulnerable to joint damage. If you begin pregnancy with a normal BMI, aim to put on about between 22 and 26lbs (10-12.5kg). If you have a raised BMI before getting pregnant, speak to your GP or midwife for advice about how much weight you can afford to gain (or even lose) during pregnancy. Below are a few of the most common muscular-skeletal issues seen in pregnancy.

 

Pelvic girdle pain (PGP)

This used to be called ‘symphysis pubis dysfunction’. The increased laxity and instability of the pelvic joints cause pain all around the pelvis, which can range from mild aching after sitting still, to considerable disability requiring use of crutches or a wheelchair. Some studies have found that, whilst the general pregnant population has a 7% incidence of PGP, this is increased to 26% in the woman with hEDS/HSD. Symptoms can begin earlier in the woman with hEDS/HSD and it may take a lot longer for them to disappear after birth. Some women with hEDS/HSD continue to suffer with PGP for many years after childbirth and considerable support may be needed. Try the following:

◆ Ask to be referred to a physiotherapist who can offer help and support by teaching exercises, providing specialist pregnancy belts and suggesting simple lifestyle changes to minimize pelvic instability. It may be wise to request referral early in pregnancy in order to get advice to reduce the

likelihood of PGP occurring.

◆ When sitting and lying, try to keep your legs parallel and hip width apart. Avoid crossing your legs or sitting at awkward angles.

◆ Avoid standing on one leg for dressing (sit on the bed or chair to put on socks).

◆ Keep legs comfortably together when moving in and out of the bath, bed and car or when turning over in bed.

◆ In bed, rather than simply putting a pillow between your knees (this is often recommended but can make pain worse), put a pillow or two between the whole length of your legs – there are ‘body pillows’ sold specifically for this purpose and the investment might be worthwhile.

◆ Put a warm hot water bottle or heat pad on the lower back.

◆ Use a TENS machine on the back.

◆ Take regular paracetamol under the supervision of your GP.

◆ Avoid lifting heavy objects.

◆ Change position regularly to reduce stiffness and aching.

◆ Maintain your usual exercise routine. There seems to be particular benefit for those with hEDS/HSD from hydrotherapy.

N.B. Many women find that the naturally occurring endorphins and adrenaline of labour reduces their PGP pain significantly during the birth, but do ensure that your birth plan details your PGP symptoms as well as your hEDS/HSD so that your caregivers can protect your pelvic joints. You need to detail how far you can move your legs apart without pain, request that vaginal examinations are done with you lying on your side with leg-parting reduced and to avoid use of lithotomy poles (stirrups) if at all possible.

 

Back pain

This may occur with or without PGP. The strategies used to alleviate PGP are also relevant for non-PGP backache. In addition, take extra care with posture. There is a natural lordosis (swayback) in pregnancy and good posture can help to correct this just enough to reduce over-stretching of the ligaments. Try to tuck your tail-bone under as you sit and walk, and ‘walk tall’. Consider the chair you use in the house and at work and ensure that your feet can rest comfortably on the floor. Use a small cushion for your lower back and raise your legs on a comfy stool if it helps. Wear well-fitting shoes with a low heel and remember to insert any orthotics. You may find that your feet get bigger during pregnancy so get properly measured.

Pace your activity and avoid overdoing it on good days. This may mean asking for extra help with household tasks and sitting down for ironing, washing up etc. Take great care when lifting – avoid if possible but, otherwise, bend your knees, keep your back straight and bring the item to be lifted in close before picking it up. Avoid twisting as you lift. Most NHS physios run back care classes for pregnant women and your midwife should have details of these.

 

Pain management

The increased laxity in the hEDS/HSD joints and tissues can lead to the initiation or increase in pain that should not be ignored. Whilst back pain and PGP are probably the most obvious musculoskeletal problems, necks, knees, ankles and feet, as well as other joints, can suffer too. Speak to your GP about your current pain medication so that it can be changed to be both safe in pregnancy as well as adequate to keep you comfortable. Most people with hEDS/HSD have preferred coping strategies for their daily aches and pains. Here are a few other suggestions:

◆ Massage

◆ Warm baths

◆ Heat packs (do not put these on your tummy)

◆ Fidgeting

◆ Gentle, daily stretching without over-stretching

◆ TENS

◆ Hydrotherapy

◆ Pregnancy Pilates

◆ Mindfulness meditation

◆ Distraction (music, reading, crafts, cooking, etc)

 

Proprioception

Many with hEDS/HSD have very poor proprioception (joint position sense) which can lead to stumbles, trips and falls. Pregnancy hormones can make it even harder to work out where your body is in space and you should take extra care on stairs and on uneven ground.

 

Heart palpitations

Many people with hEDS/HSD are used to heart palpitations and ‘flutters’. These may increase, or become apparent for the first time, in pregnancy. Tell your GP who can arrange a quick ECG to ensure that there is nothing to worry about. The palpitations should settle back to normal-for-you after the birth as hormone levels settle down.

 

Breast changes

Most (but not all) women find that their breasts grow during pregnancy due to hormonal changes. The hyper-elastic skin in the woman with hEDS/HSD means that extra support is important. A properly fitted bra should include wide shoulder straps and supportive material without seams over the sensitive nipple area. If you are used to and prefer underwired bras then carry on wearing them as there is no evidence that they cause damage to the pregnant breasts. However, many women find that sports or yoga bras have the firm stretch and wide straps that give comfort and support.

There is no need to ‘prepare’ breast and nipples for breastfeeding. After birth your breasts will become full and heavy as your milk ‘comes in’ around the third or fourth day. It is safe to take paracetamol and Ibuprofen for pain and it can help to wear a very soft but supportive bra, even in bed. Cold compresses can reduce the engorgement and there are gel packs that can be bought for this job.

N.B. Savoy cabbage leaves have only been shown to work as long as they are cold and only work because they are cold – in other words, it is the cold rather than the cabbage that soothes the swollen breasts!

The best way to avoid engorgement is to allow your baby completely free access to suckle. Women that feed lying down and freely for the first week seem to have fewer problems with feeding and supply.

 

Stretch marks

Stretch marks are caused by over-stretching of the middle (dermal) layer in the skin. Some people with hEDS/HSD are more prone to stretch marks and pregnancy is a common cause. There is currently no proven preventative treatment and there is no way of getting rid of them once they appear. However, massaging the skin with a good oil such as grapeseed oil or jojoba oil is relaxing and may ease some of the itching associated with stretch marks. They fade in time.

 

Dietary deficiencies

Good nutrition is very important during pregnancy and your midwife should have given you advice about diet and appropriate supplements at your first appointment. It is worth considering that people with hEDS/HSD who also have underlying gastrointestinal problems causing malabsorption might be more prone to nutritional deficiencies. Furthermore, various medications can inhibit the absorption of nutrients: for instance proton pump inhibitors (PPIs) can inhibit the absorption of iron and vitamin B12, whilst gabapentin, pregabalin and certain other drugs can inhibit the absorption of other nutrients. Always tell your midwife and GP about your gastrointestinal issues and current medications so that the correct blood tests can be taken in order to assess your nutritional wellbeing. If you are found to be deficient in any vitamins or minerals, your GP will prescribe the appropriate supplements. Never supplement in pregnancy without medical advice.

 

Mental Health

It has been noted that people with hEDS/HSD appear to be more prone to anxiety and depression. The reasons are probably multi-factorial. It is essential that mental health is monitored and, where appropriate, treated in pregnancy to reduce the likelihood of post-natal depression. The likelihood of both antenatal and postnatal depression are increased when there is a previous history of mental health problems.

Your midwife will ask you about your past and current health so do tell her about your mental health so that she can support you and help you to self-monitor.

To help you maintain good mental health, here are some other things to try:

◆ Eat healthily and regularly

◆ Get out in the fresh air every day

◆ Take regular gentle exercise such as walking, swimming or pregnancy Pilates

◆ Avoid alcohol

◆ Sit in a sunny spot or in bright daylight for half an hour every day

◆ Learn mindfulness meditation and consider downloading an app for pregnancy mindfulness

◆ Set a regular going-to-bed and getting up time

 

Labour

Women with hEDS/HSD should be very aware of the following issues and consider mentioning them in a detailed birth plan.

 

Pre-labour spontaneous rupture of membranes

Due to the fragility of the connective tissue, those with hEDS/HSD are more prone to their membranes (‘waters’) popping before being in established labour. Contrary to popular belief, the membranes do not generally pop until women are well into their labour and often not until baby is actually being born. Women with hEDS/HSD may find that their membranes pop before any labour signs and even before 40 weeks. It is worth discussing this phenomenon with your midwife and asking about your local hospital guidelines in this event. Many hospitals advise induction and antibiotics if membranes pop and labour doesn’t begin within a day or two. The evidence for this is mixed and, if you are concerned, ask to meet with your local consultant midwife. There is some evidence that, because the membranes ‘belong to’ baby rather than mum, if a baby has hEDS/HSD the likelihood of pre-labour rupture of membranes is increased.

 

Epidural

It is not uncommon for those with hEDS/HSD to have a poor response to lignocaine. As this is the drug used in epidurals, your midwife needs to know if you do not get relief from lignocaine at the dentist or if you believe that you are or may be insensitive to it. She may refer you to the hospital anaesthetist antenatally for assessment.

 

Anaesthesia

Due to the dysautonomia (malfunction of the involuntary part of the nervous system) apparent in many people with hEDS/HSD, general anaesthetics can cause a significant drop in blood pressure. If you need an anaesthetic for any reason, let your anaesthetist know that you have hEDS/HSD.

 

Precipitate birth

Although the ‘warm-up’ phase of labour (from 0 to 4cm dilation) may well last as long as that of an unaffected woman – up to a few days and nights in a first pregnancy – women with hEDS/HSD are more prone to a precipitate (very fast) active labour. As good as this sounds, it can be intense and frightening if one is unprepared or feels disbelieved. Let your midwife know that you may labour very fast from 4cm and that your caregivers need to monitor you carefully and take heed if you want to push suddenly.

 

Malposition of baby

Due to the lax ligaments and joints within the abdomen and pelvis, babies of women with hEDS/HSD may come through the pelvis and be born in a less standard position. They may come through ‘back to back’ or with their heads at an angle (asynclitic) or even with their head facing side to side in your pelvis. Because of the joint laxity, whereas unaffected women may struggle to birth their babies in unusual positions, women with hEDS/HSD may have no problem pushing their baby out ‘facing the wrong way’! Caregivers need to be alerted so that they do not feel the need to use forceps or ventouse too quickly if they discover your baby in a less common position. The author happily birthed her first son with the head facing side to side which is usually deemed ‘impossible’!

 

Bleeding

Because of the fragility of the cell membranes, women with hEDS/HSD can be more prone to bleeding after birth. Usually this is just ‘more than usual’ but it may be a lot more (post-partum haemorrhage). If your caregivers are alerted to this increased possibility, they can monitor carefully and make provision for extra care during the delivery of the afterbirth (placenta) when heavy blood loss is more common. If you do start to bleed more heavily than is usual, your midwife may suggest that you have an injection of syntometrine to help stop the bleeding.

 

Perineal tears

Your perineum is the area between your vagina and your anus and is the skin that gets stretched open as your baby is born. Women with hEDS/HSD are more prone to tearing and may take longer to heal. Here are some tips.

◆ Perineal massage: the jury is still out as to whether doing regular massage of the perineum helps to prevent tearing but women report being better prepared emotionally for the sensations of the head being born when they have massaged regularly antenatally. Use a simple massage oil and be gentle – the hEDS/HSD tissues are more prone to bruising.

◆ Don’t push too soon: request that you are not encouraged to push until you have a natural urge. The urge to push is a reflex and so will ‘kick in’ when you are ready to push. Waiting until this time rather than trying to push before you are ready will allow your baby to come down a little more slowly, allowing for a gentler stretching of tissues.

◆ Ask for extra lignocaine: if you know or suspect that you are insensitive to lignocaine (local anaesthetic) let your caregiver know BEFORE suturing starts. Extra lignocaine can be used both in and on the delicate tissues to reduce the sensation of suturing.

◆ Avoid dissolvable stitches: the hEDS/HSD skin can take longer to heal and dissolvable stitches may dissolve before helping is complete. Talk to your midwife antenatally and ask her to request silk sutures to be put by for you. These are soft and comfortable and can be left in until healing is complete. A highlighted note should be put in your maternity records detailing that you are to be sutured using your silk and where the sutures are located in the hospital. You may be given them to look after until birth.

◆ Keep your sutures clean: daily bathing or showering without using soaps and creams on your perineum can reduce the risk of infection. There is no evidence to support the use of essential oils such as tea tree, but a cold gel pad in your pants may soothe and reduce bruising and swelling.

◆ Do your pelvic floor exercises: these simple exercises are taught to all pregnant women. Most forget to do them. DON’T FORGET! They can help your tissues return to normal after birth and reduce the likelihood of urinary and faecal incontinence long-term.

◆ Take simple painkillers such as paracetamol and ibuprofen regularly to reduce pain and inflammation and so encourage you to do your pelvic floor exercises.

 

Wounds

As discussed above, the hEDS/HSD tissues need extra care if torn or cut. Ensure that you speak to your surgeon if you are expecting to have a caesarian section (C/S) so that she/he can adapt accordingly as different suturing material may need to be used.

You may be offered codeine following a C/S. This can cause constipation and you will need to adjust your diet and fluid intake accordingly.

You may be advised to feed your baby underarm (‘rugby ball’ method) following a C/S but this can be tricky and impractical as well as unnecessary. See below for suggestions on feeding and baby care.

Sneezing and coughing can feel sore after a C/S and this can be helped by holding the wound securely with your hands. Your hospital physio will talk to you about how to move and lift after a C/S.

 

Baby care

It is easy when you are focussing on caring for your new baby to forget about protecting your own body and joints. Think about your home environment before the birth and make simple adjustments to make life easier, more comfortable and safer for you.

 

Changing nappies

Make sure that everything is close to hand before you start so that you do not have to twist and reach. Use a changing table at a comfortable height or ask to be shown how to change your baby on your lap. If your proprioception is poor, you may feel safer sat down with your baby securely held on your lap. This is easy and can be done out and about too so is a trick worth learning!

 

Bathing

Leaning over a bath to use a baby bath is not good for your back so consider bathing your baby in a clean sink. As with changing nappies, ensure that everything is ready and close to hand before starting. If you struggle to hold things securely, consider buying a special seat to lie baby on in a large sink or baby bath. If you must use a baby bath then look for one that sits across the bath so that bending is minimised or put it on the kitchen work surface to make filling and emptying easier. If there is another adult in the house at bathtime, consider taking the baby into the bath with you. This is a favourite with many mothers and enables them to feed baby at the same time as getting both mother and baby washed during some skin-to-skin time!

 

Feeding

Most UK midwives teach a way of holding and feeding a baby that is difficult, uncomfortable and unsustainable for women with hEDS/HSD. Using positions that require holding for long periods of time with hands and raised elbows can lead to pain and nerve entrapment and should be avoided. The simplest positions are the comfiest for both mother and baby and it is worth speaking to a specialist midwife with a knowledge of hEDS/HSD before baby is born so that you know what to do from the very first feed. Remember that your baby has evolved to have all the necessary reflexes and that trying to help too much can just interfere with these. Using a simple cuddle hold with baby on your lap, well under the breast and looking up at you allows baby to lead the way. Keeping your arms in their natural place and bringing baby in very close makes it easier for baby to follow the natural reflex to reach up and take your breast. There is no need to ‘look for the big mouth’ as this will prevent your baby feeling your breast which is the trigger for the rooting and latching reflex. Take advantage of the extra-long arms enjoyed by many of those with hEDS/HSD and hold baby on your forearm rather than your hand. This will prevent wrist pain and carpal tunnel syndrome. Ask to be shown how to hold your baby hands free if wrist or hand pain is an issue for you. There is no need to use a special chair, sitting position or cushion. Simply find a position that is comfy for you – sitting, lying or standing depending on where you are at the time, and then feel free to change position if you get fidgety or uncomfortable after a while. As long as you keep baby close to you as you move, you can shift around as much as you need! The majority of pain medications are safe when nursing but ask a lactation specialist if you are unsure.

 

Carrying

Your baby will need to spend much more time in arms than you expect and, during the first 12 weeks, don’t expect your baby to settle out of arms very much at all. This is completely normal and is an in-built protective mechanism to keep your baby safe. However, this can cause pain in women with hEDS/HSD and so it is worth thinking about how your baby can be carried before the birth.

 

Slings

Using a sling worn close against the body can help to balance the weight evenly without putting strain on the back and arms. There are many ‘close carriers’ on the market but these can be pricey. You can make a very cheap and simple ‘ring sling’ from a regular shawl (look on the internet for instructions). As your baby grows, you can ‘wear’ him/her on your back. Of course, if you have a partner or friends and family around, you can share the work of carrying.

 

Prams, pushchairs and car seats

There is a huge array of baby equipment, however many items are heavy, cumbersome and not geared towards those with hEDS/HSD. Buy minimally and wisely. Think about the weight of the kit and how easy it is to fold it down and put in the car. Think about where you can store it when not in use that is easy for you to access and doesn’t require lifting. Prams and pushchairs often have adjustable handle height so check before buying that it is right for you and consider getting one that allows baby to face you and up high to minimise bending to get her/him in and out. Also ensure that you can easily manage the clasps and locks and that getting them in and out of the car is safe and comfy for you.

The same is true of car seats – some are lighter and easier than others so borrow a friend’s baby, if possible, and test before buying. Keep the car seat on a table or work surface so that you are not bending to get baby in and out or lifting from too low down. It might be that a car seat that clips into a pram frame is easiest so that you do not have to carry baby from the car to the house. It may be a careful balance and compromise between weight and ease of handling and folding. In view of the cost of these items, it is worth spending plenty of time looking for something that is right for you and your needs.

 

In Conclusion

HEDS/HSD can present many and varied challenges during pregnancy, birth and parenting. Some of these are exacerbations of issues that unaffected women face, and some are particular to those with hEDS/HSD.

A considered and well-planned approach, together with the help of members of your healthcare team, can help to make it a joyous experience with minimal complications.

Peer reviewed by: Donna Wicks, Registered Nurse and CEO / Senior Medical Liaison Officer, HMSA

Date of last review: 01/10/2017

Date of next review: 01/10/2020

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