Ehlers-Danlos syndromes (EDS) are hereditary collagen disorders. EDS may affect the skin, ligaments, muscle, blood vessels, and organs since collagen is a major component of connective tissue and is found throughout the body. The hypermobile, classical, and vascular forms are the most common, with hypermobile EDS (hEDS) being the most common. Many people with hEDS go undiagnosed for years, experiencing pain, anxiety, and a variety of other symptoms. HEDS may also affect the autonomic nervous system, resulting in dysautonomia and autonomic dysfunction, including dysregulation of all involuntary bodily functions, such as blood pressure, heart rate, temperature, breathing, and balance.
Many people with EDS report a rise in the number and severity of symptoms during pregnancy due to hormonal changes. Some problems are unique to pregnancy and birth that may pose additional challenges. Midwives, GPs, and other healthcare professionals can better serve and treat their patients with EDS if they know the problems they can face.
Minor pregnancy complications
Blood vessels widen and bulge due to the weakened collagen in EDS, contributing to a higher frequency of varicosities. Progesterone levels rise during pregnancy, softening the smooth muscle of the blood vessels even further. In the patient with EDS, varicose veins, hemorrhoids, and vulval varicosities may occur or worsen, and basic self-help measures should be recommended at the first appointment.
Varicose veins (also known as spider veins) are a type of vein that Encourages your patient to stick to his or her regular workout routine. Water exercise (swimming, hydrotherapy) and gentle walking are especially beneficial, and many physiotherapists offer hydrotherapy courses. Circulation can be improved by elevating the thighs, avoiding crossing the ankles or knees, and doing passive leg exercises. Support tights will provide relief from aching veins.
Internal and external hemorrhoids (piles) can be alleviated by avoiding constipation. Regularity of bowels can be supported by a balanced diet rich in fiber (fruit, vegetables, grains), fluids, and gentle exercise. Pelvic floor exercises should be taught and encouraged by your patient to do them daily to help with circulation. If your patient is frail, seek alternatives to iron tablets because they can cause constipation. Various cooling gel items on the market may be useful in soothing the perineum after childbirth. Self-help solutions aren’t always enough, and a prescription for effective creams or suppositories is needed in some instances.
Reflux causes the burning, acidic sensation in the esophagus. Reflux is common in people with EDS, and symptoms can worsen during pregnancy. Spicy foods, citrus foods, alcohol, caffeine, and milk may all exacerbate symptoms; however, consuming small, daily meals, drinking plenty of water (to dilute the acid), avoiding late-night meals, and sleeping on your left side can all help alleviate symptoms. If the symptoms continue, an antacid prescription should be considered.
The additional slackness of blood vessels in EDS can worsen symptoms, a common pregnancy symptom. Keep an eye on your patient and advise her to keep her legs higher than her hips at rest and stop crossing her ankles. Daily passive leg exercises and support tights can help to increase circulation and reduce edema. Carpal tunnel syndrome symptoms in women with EDS may appear earlier and be more intense, causing tingling and numbness in the fingers and, in some cases, radiating pain up the forearm. A physiotherapist can give splints and basic exercises to alleviate pain and swelling.
Vomiting and nausea
EDS is a common complication of pregnancy that is difficult to treat. Women with EDS may be more vulnerable to elevated progesterone levels, which may cause nausea, vomiting, and vertigo. The majority of proposed therapies are not effective in the research. Maintaining a healthy blood sugar level, on the other hand, tends to be beneficial. Suggest that your patient often eats and in small amounts and maintains a healthy blood sugar level by consuming a balanced diet that includes both basic and complex carbohydrates. If middle ear symptoms are present, avoiding foods and fluids that cause nausea and, if middle ear symptoms are present, minimizing abrupt head movements and sitting peacefully at the worst times of day can help. Nausea and vomiting usually subside about the 16-week mark, but for those suffering from progesterone-induced middle ear symptoms, continued treatment may be required during the pregnancy. If the symptoms are serious, hospitalization for medication and rehydration may be required.
Many people with EDS experience headaches daily. Hormonal shifts and a faster metabolism may increase the frequency of headaches in all pregnant women, and those with EDS can need additional help. Can fluid intake and taking basic pain relievers like paracetamol can be extremely beneficial. Nasal congestion, which is common during pregnancy, can cause headaches. As a simple self-help strategy, steam inhalations and sniffing oils like eucalyptus in common remedies (Vicks and Olbas Oil) can be recommended. Your patient must understand that if her headache is accompanied by flashing lights, nausea, or is not relieved within an hour of taking paracetamol, she should seek immediate medical attention.
Tinnitus is a common symptom of EDS, and it is caused by the instability of the bones in the middle ear. Extra progesterone in the bloodstream may aggravate the problem.
People with EDS often have a poor sleep, making it difficult for them to get regular deep sleep. Painful joints, tinnitus, palpitations, and poor thermoregulation can interfere with sleep. Taking simple pain relievers before bedtime, using light bedding and a cool fan, and sticking to a regular bedtime schedule can all help your patient get a good night’s sleep.
The majority of people with EDS claim their pain problems started during their first pregnancy. Likely, the increased laxity experienced during pregnancy doesn’t settle properly after birth.
In either case, women with EDS should take extra precautions to protect their joints during pregnancy and after delivery. Early referral and frequent visits with a specialist physiotherapist can be extremely beneficial in assisting your patient in remaining mobile and that joint harm. Symptoms can be minimized and treated with careful monitoring of painkiller use and timely appointments with other professionals such as podiatrists and occupational therapists. It’s also important that your patient doesn’t gain too much weight to avoid putting more pressure on their joints. The following are some of the most common musculoskeletal issues during pregnancy.
Pain in the Pelvic Girdle (PGP)
This was also known as symphysis pubis dysfunction. Pain all over the pelvis is caused by increased laxity and stiffness of the pelvic joints, ranging from moderate aching after sitting still to severe impairment requiring crutches or a wheelchair. According to some reports, while the general pregnant population has a 7 percent incidence of PGP, women with EDS have a 26 percent incidence. Symptoms in women with EDS may occur sooner, and they may take a long time to disappear after birth. Some women with EDS continue to have PGP for several years after childbirth, necessitating a lot of help. Consider and discuss the following points with your patient:
- Referral to a physiotherapist who can assist with workouts, have specialized pregnancy belts, and recommend simple lifestyle adjustments to reduce pelvic instability. To minimize the risk or severity of PGP, it may be prudent to refer early in pregnancy.
- Legs should be parallel and hip-width apart while sitting and lying. Avoid sitting with your legs crossed or at an uncomfortable angle. N.B. Since the patient with EDS may have low proprioception, she may need assistance from a companion to remind her when uncomfortable.
- It’s best to stop standing on one leg when dressing (sit on the bed or chair to put on socks).
- When getting in and out of the bath, bed, or vehicle, or turning over in bed, keep your legs securely together.
- Use a pillow or two the entire length of your legs in bed or while sleeping, or a special “pregnancy pillow.”
- Applying a warm hot-water bottle or heat pad to the lower back will provide adequate pain relief without drugs. Remind your patient not to put anything on her stomach because she’s pregnant.
- You can use a TENS machine on your upper or lower back.
- Under the care of a doctor, regular paracetamol use is currently considered a safe method of long-term pain relief.
- Lifting large items should be avoided.
- Changing positions regularly will help to relieve stiffness and ache.
- Sticking to their daily workout schedule. Hydrotherapy seems to be particularly beneficial to people with EDS. N.B. Several women report that the naturally occurring endorphins and adrenaline of labor greatly decrease PGP pain during delivery. However, your patient’s maternity notes and birth plan must provide information about her PGP symptoms and EDS so that any birth attendants can protect her pelvic joints.
- Measure and log how far your patient can shift her legs apart without pain before term, ask for vaginal exams on her side with leg-parting minimized, and avoid using lithotomy poles if at all possible.
This can happen with or without the use of PGP. The methods used to treat PGP backache are also applicable to non-PGP backache. Also, remind your patient to take extra precautions with their posture. Encourage the EDS patient to gently tuck her coccyx under as she sits and walks and to “walk tall,” as the normal lordosis of pregnancy can be corrected only enough to avoid overstretching the ligaments. Her weak proprioception can cause her to over-adjust, and your input may be useful in gently correcting her lordosis.
Asking for extra support with household chores and settling down for ironing, washing up, and other activities can help to relieve pain and fatigue.
Lifting should be avoided if at all practicable, but if it is necessary, knees should be bent, backs should be straight, and the object to be raised should be carried in close before being picked up. When lifting, your patient should stop twisting.
Most NHS physiotherapists provide back care courses for pregnant women, and rather than waiting for complications to occur, the patient should be referred as soon as possible after booking.
EDS can result in a loss of proprioception, leading to stumbling, slips, and falls. Your EDS patient will also be aware of their propensity for stumbling and need guidance about avoiding stumbling on stairs and uneven floors.
Palpitations in the heart
Heart palpitations and ectopic beats are common in people with EDS. During pregnancy, these symptoms can worsen or appear for the first time. An ECG can be performed to rule out any potential problems. After the birth, her palpitations should return to normal levels as her hormone levels stabilize.
Due to hormonal shifts, most (but not all) women’s breasts develop during pregnancy. The hyper-elastic skin of women with EDS necessitates additional assistance. Broad shoulder straps and comfortable material without seams over the sensitive nipple region should be used in a properly fitting bra. If your patient is used to wearing underwired bras and enjoys them, she should be assured that there is no proof that they damage pregnant breasts. Many women, however, prefer sports or yoga bras because they have a firm stretch and large straps that provide comfort and support.
Breastfeeding does not require any ‘preparation’ of the breasts or nipples. Normal paracetamol and ibuprofen can relieve the pain of engorgement after birth as the milk “comes in,” and wearing a very comfortable yet supportive bra, even in bed, can provide relief. Demand-feeding and over-the-counter cold compresses will also help to relieve engorgement. N.B. even though many caregivers still prescribe them, RCTs have shown that savoy cabbage leaves only work for as long as they are cold and only work when they are cold – in other words, the cold, not the cabbage; soothes swollen breasts!
It’s worth remembering that women who eat while lying down and unrestricted for the first week tend to have fewer feeding and supply issues.
Overstretching of the dermal layer of the skin causes stretch marks. Stretch marks are more common in people with EDS, and pregnancy is a common cause. There is currently no proven preventative medication, and once they emerge, there is no way to get rid of them. Massage the skin with a good oil like grapeseed or jojoba oil; on the other hand, it is soothing and may help relieve some of the scratching associated with stretch marks. They fade with time.