Vascular Ehlers-Danlos Syndrome

Learning how to effectively manage your condition and maintaining a support team can reduce risk and help you learn how to live with vEDS. Many people who have vEDS live a completely normal life and are physically fit. However, there is always a risk of an unpredictable problem occurring such as blood vessel or organ rupture.

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Activities

There are many activities that can help manage your condition and help you lead a healthy life. But there are also some types of activity you should avoid.

Some activities that should be avoided due to having a higher risk of causing injury and stress include:

• Contact sports such as fighting sports (e.g. boxing, wresting, martial arts) and rugby

• Activities/sports that involve sudden exertion

  • Weightlifting (including lifting or pushing heavy objects in day to day life)

  • Sprinting

• Exercising to the point of exhaustion

• Playing brass instruments as it increases pressure put on the blood vessels and lungs

• Scuba diving

Despite avoiding these activities, there are still many that you can partake in and enjoy. As long as an activity does not cause exhaustion, muscle strain, or constant increased blood pressure and heart rate, they should be okay to do, although to be sure you should check with your support team. For some activities you may need to wear protective clothing, and be aware of how to reduce strain on your body.

Low-intensity activities are always encouraged to maintain general health and to enjoy the social and mental benefits. Some of these activities to be performed in moderation could be:

• Walking/hiking

• Steady jogging

• Cycling

• Swimming

• Pilates

• Other steady aerobic exercise

• It is suggested that activities should be on well-cushioned surfaces (e.g. gym)

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Healthcare

Building a care team is an extremely important part of managing your condition. A care team should comprise of a primary care physician, a vascular surgeon, and general surgeon, a geneticist, and a cardiologist. It is important to see a cardiologist on a regular basis (annually or more often is recommended) to monitor your blood pressure and vessels, as well as to discuss management and medication. Your care team will ensure that your quality of life is affected as little as possible.

It is recommended to wear a medical bracelet, a medical alert disk, or carry a EDS passport outlining your condition to inform any bystander or medical professional about your diagnosis in the event of an emergency. These should carry information about what the conditions is, what the main risks are, your blood group, and details of your medical support team. Because vEDS is a rare condition, it is not uncommon for medical staff to not know about the condition. It is important to inform them of the risks of certain medical interventions. If you are unable to provide this information, a family member or your medical alert disk will be able to. It is important to inform your local A&E department about your condition so they have records and protocol if you were to come in.

If arterial, uterine, or digestive complications are observed, immediate hospitalisation and observation is advised. Any unexpected pain should also be an indication to seek immediate medical attention.

You should avoid having any kind of surgery or invasive treatments, tests and imaging unless completely necessary as it may cause damage to your fragile tissues and cause serious complications. This includes colonoscopy, arteriography, x-rays, and any elective surgery.  If you have to have life-saving surgery, it is important that the surgeon and medical team appointed to you are aware and informed about your condition, so they can handle your tissue and organs appropriately. If you experience multiple perforations in the colon and/or intestine, it is often treated by a colostomy or an ileostomy.

There are also certain medication-types that should be avoided as they interfere with coagulation. Your medical team should know which types of medication should not be used, so you should be prescribed them, but it is useful to know yourself just in case.

vEDS often comes with other conditions and illnesses, as the fragility of your tissue can cause complications. It is important to be aware of other conditions that you have an increased risk of getting. This will allow you to recognise them if they occur and get the correct help.

 

Children

12-24% of people with vEDS have major complications before the age of 20 (Pepin et al., 1999), so even if your child does not display any serious symptoms now, they can at any time. Once your child has been diagnosed, it is important to inform all those that may care for them in case of an emergency. This includes:

• Schools/teachers

• Childminder/babysitter

• Activity leaders (e.g. sports coach, scout leader)

Children with vEDS can struggle in schools with things that don’t seem obvious, such as carrying heavy books/backpacks, sitting for long periods of time, writing for long periods of time, and having to wait to use the toilet. Children with the condition can have poor stamina, as well as bladder and digestive issues, this can cause them to be fidgety, have poor concentration, and get tired quicker than other children. Because of this, it is important the school staff are aware of the condition and know how to appropriately accommodate the child. However, there can be difficulty in doing this without the child feeling labelled as different and singled out. It is important for the child to live a normal life and participate with all activities, including physical education, just as long as the activity can be altered to reduce stress and risk for the child. Often, a care plan will be organised with the school to make sure the child gets the best care and education possible.

 

Pregnancy

Pregnancy can be dangerous for those with vEDS as it puts your body, organs, and cardiovascular system under intense pressure. The fragility of your organs and blood vessels increases the risk that pregnancy already has greatly, with the maternal mortality of women with vEDS being 5.3% and 50% of women having complications (Pepin et al., 1999). While many people with vEDS still decide to have children, it must be kept in mind that there are other options such as adoption or surrogacy.

If you are a woman with vEDS who is pregnant, it is vital to ensure your pregnancy team (midwives, obstetrician) are well informed about vEDS and what this can mean for your pregnancy. They must know what to do in case of an emergency situation, and they must be well-informed enough to advise you. They will most likely put you in a high-risk obstetrical program.

It is recommended that maternity leave should be started at 30 weeks, or earlier if recommended. You should also rest as much as possible during this time as being pregnant can put a lot of strain on your body, which can increase the chance of blood vessel or uterine rupture (especially in the last 3 months).

Giving birth to your child can be difficult if you have vEDS. Firstly, there is an increased risk of premature birth due to the fragility of the tissue in the membranes surrounding the child. Secondly, there is increased risk of tears in the birth canal during delivery. Some may suggest giving birth via cesarean section to avoid some of these risks, but the benefits of doing this has not yet been studied.

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References:

Bowen, J., Tocher, J. (2016) Vascular Ehlers-Danlos syndrome. Available at: https://www.ehlers-danlos.org/information/vascular-ehlers-danlos-syndrome/ (Accessed: 03 November 2018).

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Ehlers-Danlos Support UK (unknown). Available at: https://www.ehlers-danlos.org/ (Accessed: 24 October 2018)

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Germain, D. (2007) ‘Ehlers-Danlos syndrome type IV’, Orphanet Journal of Rare Diseases, 2(32). Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1971255/ (Accessed 12 November 2018)

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Pepin, M., Murray, M. and Byers, P. (1999) [Updated 2015] ‘Vascular Ehlers-Danlos Syndrome’, GeneReviews. Available at: https://www.ncbi.nlm.nih.gov/books/NBK1494/ (Accessed: 4 November 2018).

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NHS (2016) Ehlers-Danlos Syndromes. Available at: https://www.nhs.uk/conditions/ehlers-danlos-syndromes/ (Accessed: 24 October 2018)