What do you need to bear in mind if you have ampullary epidermolysis or you are caring for someone who does?

The best thing is if the patients, their families and their caregivers receive comprehensive health education, especially when they are first diagnosed, during the baby’s first few days, when skin lesions can already begin to occur.

The education aimed at preventing the evolution and complications of the disease will be given by professionals from the following disciplines:

• Dermatology
• Plastic surgery
• Nutrition
• Paediatrics
• Rehabilitation and occupational therapy
• Community and hospital nursing
• Psychiatry
• Psychology

Skin affected by ampullary epidermolysis is very sensitive to the slightest pressure or friction, which then causes a blister to form. To avoid damage, bear in mind the following recommendations:

• Do not apply any adhesive products to the skin, such as plasters, stitches, electrodes, adhesive dressings, etc.
• Intravenous channels should be attached using gauze and cohesive bandage on top, without touching the skin, or with a silicone plaster. If pressure needs to be applied, Vaseline should be placed on the pressure point.
• The patient should always be lifted with open hands, without causing any friction or pressure.
• Their surroundings should be protected, adding cushions and pillows to avoid unnecessary blows.
• A water mattress may be used with a foam, latex or memory foam support.
• Nappies should not be too tight, and the skin should be protected from fiction using gauze or cotton.
• Clothes should preferably be cotton, as it breathes more easily and is easier to put on. Avoid rubber, zips, labels and other details that may rub the skin. Seams should be put on inside out.
• The skin should be protected from sunlight and keep an eye out for spots and freckles.
• Keep the skin as hydrated as possible.

Other related health information

• Treatment of injuries (dressings and healing materials)
• Treatment of skin complications
• Treatment and decontamination
• Treatment of nutritional and gastrointestinal complications
• Treatment of pain
• Infection control
• Rehabilitation treatment

What do you need to bear in mind if you have amyotrophic lateral sclerosis or you are caring for someone who does?

In order to improve the respiratory difficulties in patients, ventilation therapy can be used through non-invasive ventilation.

Ventilation therapy refers to breathing support using a ventilator, usually at night during sleep, to achieve:

• A decrease in respiratory effort (feeling of fatigue)
• Improved quality of life for patients with ALS

Ventilation is carried out non-invasively, by means of a patient-adjusted mask (nasal or full face) connected by a tube to the ventilator or respirator.

When patients need this therapy, the place and time it is started, whether outpatient or hospital admission, is planned in a personalised way with the consent of the patient and the person caring for them.

Education for the patient and their main carer should begin as soon as possible, both from the point of view of managing secretions and the resulting care, as well as the emotional support they need to receive. This means that during the patient’s admission or outpatient visit, the patient and their carer will be trained in:

• Correct use of a respirator
• Fitting and adjusting the mask
• Care to prevent possible skin sores
• Hygiene
• Looking after the respirator

The patient and the carer must take care to keep the airway in good condition to allow secretions to be managed. It is important to preserve the ability to cough where possible, but if coughing is no longer effective, the patient and carer will need to start learning how to use mechanical aids (cough assist or mechanically assisted cough). In certain cases secretion suction may also be used.

To improve the quality of life of patients it is important to follow the advice below:

• Nocturnal ventilation: to be carried out daily, but gradual adaptation should be permitted until the patient is able to sleep through.
• After eating, wait between one and two hours before ventilation.
• Assisted cough should be done every day in order to maintain the skills learned by the patient and their carer, which will facilitate quick resolution of the situation if required.
• All scheduled appointments should be attended where any doubts raised will be resolved.
• Possible infections should be prevented: with flu and pneumococcal vaccinations according to medical advice, by going out in the sunniest hours of the day during the winter and the least sunny in the summer, and contact with people with respiratory infections should be avoided.
  • If signs or symptoms of respiratory infection appear (fever, increase or change in secretions, breathlessness, etc.) you should consult the healthcare team immediately.

Other related health information

• Emotional support to patients and carers in ALS
• Advice for preventing sores
• Advice for patients with a tracheotomy
• Advice for patients with problems swallowing
• Documenting the patient’s wishes

What do you need to bear in mind if you have diphtheria or you are caring for someone who does?

Diphtheria is transmitted via the respiratory tract, mainly, and also by direct contact with a sick person or a healthy carrier of the bacteria. The illness may affect the tonsils, pharynx, the larynx, the nasal mucous membrane and, much less frequently, the skin or other mucous membranes. The bacteria forms a thick grey membrane with a dark red swollen area around it, which in the case of the nose and throat may obstruct the respiratory tract.

Some people may carry the bacteria in their nose or throat. If these people are vaccinated they will not develop the illness, but they may transmit the bacterium to other people via droplets produced when they sneeze or cough. The existence of carriers in countries with no cases of the illness is very rare.

A stroke is a clinical syndrome characterised by rapid development of signs of neurological involvement lasting more than 24 hours. Vascular in origin, a stroke is considered a medical emergency that requires immediate diagnosis and treatment.

The person who has suffered a stroke usually needs further rehabilitation but, in general, it is important to respect their initiative and autonomy, even if it takes them longer, and to avoid overprotection.

Recommendations and treatment for relatives and carers

Communication

• Speak naturally, using simple words and short sentences, without shouting.
• In the first phase after a stroke, ask short, yes/no questions.
• Promote conversations about every-day life and familiar situations to stimulate the patient’s language.
• If necessary, accompany conversations with gestures to facilitate understanding.
• Items such as the radio or television should be turned off to avoid distractions.
• Respect the response time: avoid showing impatience or cutting off the patient.
• In serious cases, you can use alternative means of communication, such as boards with everyday images on them. There are also mobile apps that can help.

Movement

• Do the exercises recommended by the physiotherapist.
• Move joints and avoid incorrect positions.
• When moving a patient, never touch the affected side.
• It is important that the patient be out of bed for as long as possible.
• Pillows should be used to protect zones of friction and position should be changed every four hours.
• Before the patient drives again, a specialist should be consulted.

Sleep and rest

• It is recommended to sleep seven or eight hours a day.
• Provide a quiet environment and provide a point of light, so the patient can orient themselves during the night.
• Create a day atmosphere (more active, bright) and a different, night atmosphere (quieter, darker). This helps in understanding the sleep pattern.
• Avoid letting them sleep during the day, except for a short afternoon nap.
• After a stroke, the patient may find themself more tired for a while. It is important to become more active but without forcing it.

Hygiene

• Encourage the patient to collaborate in their personal hygiene.
• Whenever possible, use a shower, instead of the bathtub, with a chair inside and a handle on the wall.
• The soap should be neutral and free of alcohol, as this can dry the skin.
• The skin should be dried properly, taking special care with folds. It should then be hydrated and protected.
• Maintain proper oral hygiene.
• Watch bony prominences to prevent pressure sores.

Getting dressed

• Wear comfortable clothes that are easy to put on and take off, such as sportswear with elastic and no buttons.
• Start dressing the patient on the affected side and undressing them on the side that is not affected. It is preferable to be seated.
• To put on trousers (pants), especially if the patient is in bed, it is easier to gather up the trousers, insert one leg at a time and then pull them up.
• Shoes should be closed, with no laces and non-slip soles, which fit the foot well. Shoehorns can make it easier to put on shoes.
• The patient feels the same degree of cold and heat as the carer, so do not give them too many layers.

Physical obstacles

• Remove obstacles such as carpets or chairs to avoid tripping.
• Adapt the home to the new situation: install bars, a taller toilet or shower, etc.
• Adapt the home to improve patient autonomy and prevent falls. For example: have a high bed (70 cm) to make it easier for the patient to get up and down comfortably.

Leisure and recreational activities

• Maintaining social life and hobbies, while respecting the limitations of the patient, improves self-esteem and avoids isolation.
• Incorporate leisure activities gradually, according to the degree of autonomy and in consensus with the patient.

Sex

• Immediately after a stroke, sexual desire may decrease, but it will gradually recover. If in doubt, consult a doctor.
• Paralysis may make sex trickier, but not impossible.
• Fear of suffering another stroke during sex is generally unfounded.
• It is important to talk to your partner about any fears or worries that may arise.

Return to work

• The return to work must be planned, together with the company, so it is gradual, both in terms of demands and time.
• After re-joining the workplace, the situation should be re-evaluated in case changes or adaptations need to be made to improve patient performance and ability.

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The treatment for Coeliac disease is to follow a strict gluten-free diet for life. A gluten-free diet should be based on a varied and balanced diet combining foods that do not contain gluten, including gluten-free cereals. Cross contamination at home should also be taken into account (making sure foods suitable for Coeliacs have not come into contact with other foods, utensils or surfaces that contain gluten) and precautions taken when eating out. It is also important to always check the ingredients list on food labels.

This sheet contains basic tips on having a gluten-free diet in a safe and balanced way.

What do you need to bear in mind if you have Coeliac disease or you are caring for someone who does?

Gluten is a protein complex found in cereals. It is made up of two proteins, gliadin and glutenin. It is important that Coeliac patients permanently remove foods containing gliadin and glutenin from their diet. This means eliminating wheat, barley, rye, oats, triticale (a hybrid of wheat and rye) and all derivatives.

Although foods must be removed from their diet, patients with Coeliac disease must follow a balanced diet, ensuring they get sufficient nutrients. To achieve this you need to incorporate a wide variety of foods with different preparation and cooking methods.

Patients with Coeliac disease should base their diet on dairy, meat, fish, eggs, fruit, vegetables and pulses, and cereals they can eat, such as corn, rice, quinoa, millet, amaranth, sorghum, teff or buckwheat. Sugar and processed foods should be ingested to a lesser extent.

• Cereals WITH GLUTEN: wheat, barley, oats and triticale
• Cereals WITHOUT GLUTEN: rice, quinoa, millet, corn, amaranth, sorghum, buckwheat and teff

Foods should be prepared as normal, avoiding cross contamination: boiled, steamed, grilled, fried or baked. Batter and breadcrumbs should be made with flour or bread that is suitable for Coeliacs.

It is important to take care with processed or packaged foods. It is harder to avoid cross contamination in processed foods.

Read product labels carefully when you are buying food that is not fresh. Some foods naturally do not contain gluten, but in their commercially available form they do, as gluten is sometimes added during the manufacturing process. To be on the safe side, it is therefore better to avoid unlabelled products, such as those bought in bulk or handmade products.

It is a good idea to have a space set aside just for storing gluten-free foods. You should also use clean cooking utensils to make sure they have not come into contact with products containing gluten.

When eating out, take precautions. It is important to make sure that what you eat has not come into contact with any food containing gluten. One example would be oils in which foods containing gluten have been fried.

What do you need to bear in mind if you care for an infant with congenital heart disease?

To improve the quality of life of infants with congenital heart disease, you are advised to follow these daily tips and routines.

Diet

• Follow a varied and balanced diet, adapting to each patient’s needs.
• Some breastfeeding babies will need to compliment their diet with milks that are high in calories or, in some cases, food will need to be given through a nasal tube to ensure the baby continues to gain weight.

Hygiene

• Wash hands often to avoid infection, especially respiratory infections.
• Clean teeth and mouth to avoid caries and infections that may result in endocarditis.
• Avoid piercings and tattoos.

Physical exercise

• Depending on the type of heart disease, the child may be able to live a normal life and take part in activities corresponding to their age.
• In particular cases, limited physical activity is recommended, especially strength training (weight lifting, rowing, etc.).
• It is important not to overprotect children with heart disease so that they can live as normal a life as possible.
• Patients and family will be told about the different foundations and associations that offer psychological and social support to people with congenital heart disease.

Consult a paediatrician if the infant has the following symptoms:

• Tiredness when feeding from a bottle or eating poorly and not gaining weight.
• Cold sweat, mainly on the head.
• Cough and congestion.
• Changes in activity: if they get tired more than normal or become more inactive.
• Vomiting or diarrhoea, as this may lead to a significant loss of fluids. Children who take diuretics are more likely to become dehydrated. In children who take digitalis (Lanacordin), vomiting or diarrhoea may be a symptom of intoxication.
• Fever, as it may indicate decompensated heart failure. It is also important to rule out a serious infection.
• Bleeding, especially in patients being treated with antiplatelets or anticoagulants.

Warning signs to go to A&E

• Pale, grey and cool skin.
• Less urine than normal.
• Agitated, very fast breathing with a lot of effort, ribs marked on breathing (pulling).
• Tachycardia, arrhythmia or palpitations.
• Significant irritability or poorliness.
• Hypoxia crises, that is, when serious episodes occur in cyanotic heart defects: the infant goes blue, breathes very quickly and is very irritable.
• Chest pain.
• Fainting or syncope.
• Complications following heart surgery. It is necessary to monitor whether the wound has signs of infection (fever higher than 38 °C) or if there are changes in the colour of the scar.
• Increased pain or the presence of pus.

What do you need to bear in mind if you are caring for someone with Asperger’s?

People with Asperger’s syndrome may have different requirements depending on their age, surroundings and the awareness that they have of their difficulties. For this reason, they need a tailor-made programme that responds to their specific case.

The aim of these customised programmes is to:

• Promote better adaptation in patients
• Develop social skills, taking into account the person’s interests and motivations
• Improve family communication
• Establish routines
• Promote cognitive development
• Tackle sensory dysfunction

Treatment

It is important to manage their development through different disciplines. These may include cognitive treatments, social skills programmes and occupational therapy for the patient. You also have to consider guidelines on how to resolve conflicts and how to manage pyschoeducational groups for families or caregivers.

In infants, from an emotional and attitudinal point of view, it is important to learn to identify the warning signs in their mood. In this way, we can prevent difficulties in anger management and low tolerance to frustration, since they are patients with a high degree of sensitivity to criticism. Avoid punishment as much as possible and establish more positive reinforcement strategies.

All these guidelines must be established in a space where the differences the child or adolescent presents are valued positively, including their limitations, but also their possibilities and positive aspects.

In adults, many of these characteristics continue, as Asperger’s cannot be cured. In any case, personalised treatment, involving family members and good communication with professionals can allow a better quality of life.

Non-oxygenated blood arrives at the right atrium via the venae cavae, from the head and arms (upper vena cava) and from the abdomen and legs (lower vena cava). This blood passes to the right ventricle through the tricuspid valve. The right ventricle pumps this blood, through the pulmonary valve, into the lungs through the pulmonary arteries, which is where the blood gets it oxygen.

This oxygenated blood returns to the left atrium via the pulmonary veins. From the left atrium it is directed to the left ventricle through the mitral valve. The left ventricle pumps the blood to the aorta through the aortic valve to distribute it to all the organs and tissues in the body.

The heart is irrigated by the coronary arteries, right and left. These coronary arteries divide into several branches to carry oxygenated blood throughout the heart tissue.

The heart contracts due to an electric stimulus triggered by the conduction system. The cardiac conduction system is made up of a series of cells that have the capacity to create this stimulus and determine heart rate. This stimulus begins in the sinus node, which is found where the superior vena cava enters the right atrium. This stimulus causes the atrium to contract. This stimulus then propagates the ventricle through another structure called the atrioventricular node. This conduction system is capable of increasing the heart rate when necessary, such as for example during exercise, when you have a fever, when you feel emotions, etc., or decreasing the heart rate when you are sleeping. This system is regulated by the action of different hormones or in response to nervous stimuli in the cardiac plexus.

The cardiac cycle has two phases: systole and diastole. In systole, the heart contracts to send blood to the major arteries and during diastole it relaxes to fill with blood to later be ejected.

Cardiac ablation is a procedure used for treating arrhythmia (abnormal heart rhythms). The child's treatment continues even after surgery and discharge from hospital.

Before they leave the hospital, the child and their family will be provided with the guidelines and recommendations for the recovery process by the medical and nursing team.

Recommendations

Personal hygiene

The child may have a shower 24 hours after the procedure. Immersion baths can be taken after 7 or 10 days, once the puncture area has completely healed.

The wound must be cleaned with soap and water every day, and a small amount of antiseptic applied, during the child’s first 3 or 4 days at home. We recommend use of 2% aqueous chlorhexidine. The area should be sealed afterwards with a fabric dressing.

Rest and activity

The child must rest at home for 3 or 4 days. They must not do any heavy exercise such as walking upstairs nor should they stand up for long periods. Once that period has passed, the child can go back to school.

They can lead a normal life, but without doing any sport for the first two weeks after the procedure. During the summer, the child can swim at the beach or in a swimming pool from 10 days after their operation.

Diet

The child can continue with their usual diet. It must be complete and varied.

The following aspects need to be monitored for the first few days:

Puncture point

Monitoring the puncture point. If it turns red or oozes fluid, or if the child has a fever, their reference health centre must be consulted about this.

It is normal for a small bruise to appear at the puncture point. If the swelling is large, this is a sign of haemorrhaging. It can cause pain and discomfort for the child. In that case, the child’s reference doctor must be consulted about this.

Temperature

If the child's temperature rises, the reference medical centre must be consulted.

Sensation of palpitations

The child may sense a stronger heartbeat for the first few days after ablation. This is normal and goes away after a few days.

The prescribed treatment and the recommendations from the cardiology and nursing teams must be followed.

When should you go to the ER?

Pain

A piercing, low-intensity pain of short duration may appear in the puncture area, back or abdomen. It usually gets better after a few days and can be controlled with conventional painkillers. It is common and not a cause for concern.

If the pain is more intense and prolonged, then the medical team should be consulted to avoid any possible complications.

Some warning signs to take note of:

• Fever above 38°C: if a fever appears after 24 hours, it could be a sign of an infection.
• Intense, sustained pain.
• Cold, pale legs.
• If the puncture point bleeds, it must be treated with a compression pad for 15 minutes. If it does not stop bleeding, the child must be taken to their reference health centre.
• Appearance of a large bruise in the puncture area or a hard swelling.