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WHAT YOU NEED TO KNOW:
Acute porphyria is a disorder that affects how your body makes red blood cells (RBC). Your body needs a chemical called porphyrin to make heme, a part of RBC that carries oxygen. Porphyria prevents your body from creating enough enzymes to control the process, and porphyrin builds up. High levels of porphyrin can cause problems throughout your body, depending on where it builds up. Low levels of heme can also cause organ damage, because your blood cannot bring the organs enough oxygen.
WHILE YOU ARE HERE:
is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.
A healthcare provider, called a dietitian or nutritionist, may talk to you about changes to your diet and proper nutrition. A dietitian can help you to increase the amount of calories your body needs. He may suggest liquids and foods for you to eat and drink.
- Eating a well-balanced diet will help you to feel better, have more energy, and heal faster. This diet may include fruits, vegetables, and dairy products.
- Special formulas or high carbohydrate foods may be added to your diet. These may add extra calories to help you avoid an acute porphyria attack.
A Foley catheter
is a tube put into your bladder to drain urine into a bag. Keep the bag below your waist. This will prevent urine from flowing back into your bladder and causing an infection or other problems. Also, keep the tube free of kinks so the urine will drain properly. Do not pull on the catheter. This can cause pain and bleeding, and may cause the catheter to come out.
Intake and output
may be measured. Healthcare providers will keep track of the amount of liquid you are getting. They also may need to know how much you are urinating. Ask healthcare providers if they need to measure or collect your urine.
is a small tube placed in your vein that is used to give you medicine or liquids.
You may need any of the following:
- Antianxiety medicine: This medicine may be given to decrease anxiety and help you feel calm and relaxed.
- Antibiotic or antiviral medicines: These medicines may be given to kill infections caused by germs, such as bacteria or viruses.
- Anticonvulsant medicine: This medicine is given to control seizures. Take this medicine exactly as directed.
- Antinausea medicine: This medicine may be given to calm your stomach and prevent vomiting.
- Blood pressure medicine: This is given to lower your blood pressure. A controlled blood pressure helps protect your organs, such as your heart, lungs, brain, and kidneys. Take your blood pressure medicine exactly as directed.
- Pain medicine: Caregivers may give you medicine to take away or decrease your pain.
- Do not wait until the pain is severe to ask for your medicine. Tell caregivers if your pain does not decrease. The medicine may not work as well at controlling your pain if you wait too long to take it.
- Pain medicine can make you dizzy or sleepy. Prevent falls by calling a caregiver when you want to get out of bed or if you need help.
- Sedative: This medicine is given to help you stay calm and relaxed.
This is also called neuro signs, neuro checks, or neuro status. A neurologic exam can show caregivers how well your brain works after an injury or illness. Caregivers will check how your pupils (black dots in the center of each eye) react to light. They may check your memory and how easily you wake up. Your hand grasp and balance may also be tested.
A pulse oximeter
is a device that measures the amount of oxygen in your blood. A cord with a clip or sticky strip is placed on your finger, ear, or toe. The other end of the cord is hooked to a machine.
You may have any of the following:
- Blood, urine, or stool tests: Samples of your blood, urine, or stool are collected and sent to a lab for tests. These will check the levels of your porphyrins. Healthcare providers may also learn more about your blood with there tests.
- Liver function tests: These blood tests check the enzymes and other substances made or broken down in the liver. Test results will tell healthcare providers how your liver is working.
- Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
You may need any of the following:
- Respiratory support:
- You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your healthcare provider before you take off the mask or oxygen tubing.
- A ventilator is a machine that gives you oxygen and breathes for you when you cannot breathe well on your own. An endotracheal (ET) tube is put into your mouth or nose and attached to the ventilator. You may need a trach if an ET tube cannot be placed. A trach is a tube put through an incision and into your windpipe.
- Other treatments:
- Glucose: Glucose may be given if your attacks are triggered by a low-calorie or low-carbohydrate diet.
- Hemin: Hemin, also called hematin, is an enzyme made from red blood cells (RBC). It works by telling the body to slow down the production of the precursors, or building blocks, that make heme.
- Hormone: Women may be given hormones, such as birth control pills. This may help menstruating women avoid an acute porphyria attack. Ask your healthcare provider for more information about hormonal therapy.
Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.
You may be weighed each day. Caregivers compare your weight from day to day to record how much body fluid you have. You can become dehydrated if you lose too much. You can have shortness of breath or swelling in your legs if you retain too much.
- Treatment for acute porphyria may cause side effects. Some medicines may even make your signs and symptoms worse. You may have a headache, rash, itchiness, or slow heartbeat. Hematin can make your veins swell and cause kidney or bleeding problems.
- Left untreated, acute porphyria can be life-threatening or damage your brain, liver, muscles, or kidneys. The earlier acute porphyria is found and treated, the better the chances of preventing future problems. Your health, quality of life, and ability to function may decrease without treatment.
CARE AGREEMENT:You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.