Chapter: Patients and relatives
Article: 2 of 7
Update: Mar 11, 2021
“With everything I am, I can't think of myself differently and, to whatever extent, my leg and the history associated with it, they are part of me. ... I try to adapt my lifestyle to my possibilities. This often means withdrawal... If I'm doing well, I throw myself fully back into life.” (D.G.)
The course and manifestations of the disease are as individual as the subjective experience of the disease and its consequences − so there are many “life stories”. And sometimes a different blueprint of the blood vessels also means a different life plan. Many positive examples show how this can be successful.
A long search for a competent physician often starts when the first symptoms appear. This is already burdensome in terms of time and the cost of long journeys, repeatedly disappointed hopes, absences from school and work, misdiagnoses and therapies. Often too much precious time passes before therapy starts.
Fortunately, finding a specialist with experience in treating congenital vascular malformations has become easier in recent years and in Germany. Available internet sites help people find a competent specialist faster. More and more frequently, general practitioners and specialists are linked to this network and thus are better able to help their patients.
Finally having a name for the disease and for their deviation from the norm is important for many patients, because it gives them back some “control”. It is important to use correct and age-appropriate words to describe or explain one’s illness and the therapy. Accepting a disease helps you to see it as just one part of your own personality, helping you to be a human being first and foremost and not a patient.
Once the final diagnosis has been made, many questions arise and sometimes a feeling of helplessness and fear arises. Almost every patient has already had various therapeutic attempts by the time an appropriate therapy is found. There is no standardized therapy. In addition, patients frequently experience recurrences and often need to stay on treatment for life.
Many patients face a dilemma when making necessary decisions about therapies. How should they decide on something they do not fully understand and cannot oversee the consequences of? In this situation it is especially important to be able to trust your doctor.
And that’s what a congenital vascular malformation means, too: waiting for palliative or curative therapies. Comparing the effectiveness of different therapies and drugs in trials costs money and requires dedicated physicians and researchers. However, in public funding as well as with company sponsors, congenital vascular malformations hardly receive any attention because they are classed as rare orphan diseases and are therefore “unprofitable”.
As expected, experiences with physicians are highly individual. In addition to very good medical care, however, patients also experience problematic aspects: Doctors don’t recognize their own competence limits, don’t deliver in time, try out their own methods or say, “There’s nothing we can do.” Over-treatment, inadequate or aggravating therapies are the result. Sometimes there is a lack of collaboration both between different disciplines and between physicians. In addition, there are few specialists for rarely affected areas (e.g., genital area).
Patients have experienced being left alone when things get difficult or their complaints are judged to be of psychological origin.
Since physicians are highly specialized, there are only a few who maintain an overview like a flight controller, coordinate the treatments and consider possible future developments. For care and consultation, patients want their individual quality of life to be included, so that the focus is not on what is medically possible, but on what makes sense for the individual.
Patients are increasingly seeking a second opinion, and more and more doctors understand this position.
Contact and communication between patient and doctor are regularly experienced as difficult. Frequently mentioned problems are long waiting times for an appointment, hardly any acute consulting hours, time pressure in appointments, lack of care and consultation after operations and in the event of accidents, and incomprehensible technical language. They give rise to uncertainty, worries and many unanswered questions.
Uncertainty about the disease and the prospects sometimes leads to anxiety. Fear is something natural and necessary and actually shows us important limitations. It can be reduced or overcome by a sense of inner security. This can be restored through trust, education and good experiences.
Affected children and adolescents are often not talked to, but about. In this context, they need education about their disease that is appropriate to their age and developmental stage so that they can process the disease-related information and integrate it into their subjective experience of the disease.
Having a vascular malformation means being different for some patients. Outwardly visible features are present in all aspects of life. For many patients, it is difficult to cope with being looked at. While it is human to look when something is conspicuous, it is not something that individuals can tolerate equally well every day, and there is also disrespectful staring and wordless gawping. Some patients therefore use special camouflage products to cover the colored areas of skin as “color for the soul,” while other patients consciously decide against it because it makes them feel strange.
Experiences differ widely. There are positive experiences with open interaction, but also negative ones: being pitied, overlooked or marginalized, being perceived as inferior, being credited with less competence and leadership skills (halo effect) and also sometimes being mobbed and insulted. Disrespectful wording or speech even occurs among some physicians.
“As a child I wanted to be able to walk like everyone else around me, as a teenager I suffered from the stigma of being different from others, as an adult I have slowly found my place, off the beaten track.” (D.G.)
Coping with everyday life with the disease continually presents different challenges to those affected: physical limitations, pain, spontaneous bleeding, fears, insecurities, uncertainties, reactions of fellow human beings and mental crises.
In addition, the hassle with medical costs, social insurance agencies and health authorities also take up valuable energy. There are the costs of doctors, therapies and medical aids (compression garments, wheelchairs, etc.), as well as applications for a disabled person’s certificate or applications for assistance at school and at work.
In the leisure sector, a lack of accessibility leads to severe restrictions.
The disease also affects non-involved areas of health and requires special, sometimes unpleasant measures, such as special anesthetic techniques or precautions.
People who are used to pain sometimes fail to recognize other pain as a warning signal, e.g., in the case of a ruptured appendix or bone marrow inflammation. Children in particular then fail to recognize their limits.
Some ways of dealing with the disease may look from the outside like ignoring it, or lack of acceptance, but sometimes it is the individual’s way of preventing the disease from dominating their life. Complaints or deteriorations are concealed in order not to put more burden on worrying parents or partners.
The development of the disease, experienced as a deviation from the “norm”, can lead to feelings of inferiority, fear of rejection and body shame. This sometimes results in hypersensitive reactions to evaluations and avoidance of situations in which the vascular malformation is visible, e.g., at the beach or in a restaurant.
“Early childhood especially shaped me, as I was persuaded that ‘I could one day live a normal life and do everything that others (i.e., healthy people) could do’. Over time I realized that this is not the case and that I have a disability that requires special treatment. I receive support from a psychotherapist to be able to change these basic attitudes and to adapt my self-demands to reality step by step − so that I don’t overtax myself further.” (M.S.)
Young people in particular are often restricted in their range of action. Instead of pushing themselves to their limits, they already have to follow rules and take care of their health with a lot of self-discipline. Not every sport is possible, not every event can be attended and sometimes even participation in school lessons and activities with friends is not possible over lengthy periods of time.
During this time, many adolescents withdraw from medical supervision. They do not attend check-up appointments or therapies and try to block out their illness in order to feel “normal”.
The risk of developing an anxiety or depressive disorder is 2 to 3 times higher in chronically ill children than in healthy children.
This time is a big challenge for the affected adolescents, young adults and their parents. The previous support from their familiar doctor and the responsibilities within the family will change. A new doctor may have to be found, new trust has to be built, and a new balance found between the demands of the disease and the goals of the young person.
Fortunately, collaborative and interdisciplinary solutions to the transition phase have been developed in recent years.
Career restrictions are: limited career choice, fewer career opportunities, lower salary with lower pension entitlements, absenteeism. In addition, there are costs for travel, stays and necessary doctor’s visits or medical aids that may in many cases not be financed by health insurance, even if insurance is available at all.
“In the meantime, I believe that people can succeed in coping with the disease in such a way that they say: Yes, I have a congenital vascular disease. But that is only ONE PART of me. How strong the disease is may often be defined by how much space we allow it.” (L.B.)
Vascular anomalies affect the entire person. Mental and social problems may be a consequence of or reaction to the demands associated with the disease. At first glance, this does not make everyday life or dealing with these problems any easier, but it does mean that every affected person and their relatives are not helpless. They can actively influence, change and improve things! Knowledge about the disease, positive ways of living and finding your own sources of energy provide ideas and strength to do so.
Once again, experiences and reports are divergent.
Many affected people see no positive experiences at all.
Others find positive experiences: priorities in life become clearer and more essential, tolerance becomes easier. The emotional connection with people providing help is great. When experiencing actual help and openness from fellow human beings, some learn to recognize and accept human reactions without judging them.
Some report an early confrontation with themselves, greater tolerance towards other problems in life, learning early to demand their own rights, and a lower susceptibility to “external life goals” even at a young age.
Dealing with the emerging difficulties in the course of the disease can lead to positive powers: developing discipline and perseverance, perceiving “oneself” with self-confidence, dealing with oneself considerately, developing a healthy self-love and very consciously using and enjoying existing freedoms.
Gratitude is experienced intensely: gratitude for people who listen, for people who take good care of you medically, for good health in other non-involved areas and for phases with a higher quality of life.
The status as a severely disabled person sometimes has advantages, such as better protection against dismissal, easier working conditions, subsidized car purchases or home improvements.
Some people also experience patient support groups and interaction with other affected persons as a positive help in life. Friendships have developed in such groups and during clinic visits. Through personal involvement in the patient support group, it is possible to actively stand up for your own interests and to improve medical treatment and everyday life for yourself and others. This strengthens self-confidence, competence, acceptance of the disease and enables you to participate more actively in your own therapy.