Strategies to Improve Quality of Life in FAP

Familial amyloid polyneuropathy (FAP) is known to affect a person’s quality of life, particularly through the damage it can do to the autonomic nervous system, which controls and regulates without conscious thought the body’s organs. Autonomic system problems can lead to cardiovascular (heart and blood vessels), digestive, and urinary system issues, and can cause erectile dysfunction in male patients.

Here is information about how the disease might affect each of these systems, your life quality, and treatment options that could help.

The cardiovascular system

Autonomic nervous system dysfunction can lead to several symptoms related to the cardiovascular system, including orthostatic hypotension (sudden drop in blood pressure drop when moving to stand up) and heart arrhythmias (irregular heartbeats).

Orthostatic hypotension can have a large impact on your quality of life. In early disease stages, you may feel dizzy and “light-headed” when you stand after sitting or lying down. This can make you reluctant to be active. As the disease progresses, you may have a hard time sitting up without becoming dizzy. If severe, a wheelchair may help you move about without the risk of falling.

Changes in the autonomic nervous system can also lead to heart arrhythmias, such as tachycardia (increase heart rate), which can increase your risk of complications like fainting, shortness of breath, and fatigue. All have a negative impact on daily life activities.

Several treatment options are available to help reduce orthostatic hypotension. For example, wearing compression garments (like stockings) can increase blood pressure, and lessen dizziness. Increasing the intake of salt and water to retain fluids can also help maintain blood pressure, which also reduces dizziness — but this is not recommended for patients with severe cardiopathy, as it may aggravate heart failure.

Sleeping with your head 30 to 50 degrees higher than the rest of your body, and getting up slowly, can work against a suddenness of change in blood pressure and help to ease orthostatic hypotension.

Several medications may also be able to help retain fluids and increase blood pressure, including fludrocortisone, midodrine, and droxidopa. Medications or a pacemaker may help to treat heart arrhythmias.

The gastrointestinal system

Autonomic dysfunction can affect the gastrointestinal system, causing a variety of symptoms that can include gastroparesis (or delayed gastric emptying; when the stomach is slow or unable to properly pass food to the intestines), constipation, and diarrhea.

Gastroparesis can cause you to feel full sooner than usual, diminishing appetite. It can also cause gastroesophageal reflux or “heartburn,” and discomfort after meals. You may also begin to feel nauseous after eating, and possibly vomit. All of these issues can result in dehydration, extreme weight loss, loss of energy, and vitamin deficiencies that affect your quality of life.

You may also experience diarrhea, constipation, or alternating bouts of both. Diarrhea can lead to malnutrition and dehydration, which can worsen orthostatic hypotension. Uncontrolled, diarrhea also affects your social and emotional well-being.

You may be able to alleviate your gastrointestinal symptoms by making changes to your diet and meals. Eating smaller meals more frequently may help with gastroparesis. Eating more fiber-rich foods may help relieve constipation, while eating less fiber can help with diarrhea. There are also a number of medications can also help with digestive problems.

The urinary system

Damage to nerve fibers that play a role in the bladder can lead to an underactive bladder. This can cause you not to be able to empty your bladder fully, and lead to a need for frequent bathroom breaks, feelings of discomfort, and potential incontinence. Retention of urine can also cause urinary tract infections. All of these issues can impact your work, social life, and other activities.

Setting a schedule for trips to the toilet — like every two to four hours — can help alleviate urine retention and related problems. Reducing fluid intake in the evening, before bedtime, can also work against urinary problems while asleep. Several treatments can help with urination, but you may need to avoid some if you have orthostatic hypotension, as they can make the condition worse. If urine retention becomes serious, you may need a catheterization to ensure sufficient bladder emptying.

The reproductive system

Men with FAP may experience erectile dysfunction. Patients with bladder dysfunction can also experience retrograde ejaculation, or semen that enters the bladder during orgasm rather than emerging through the penis.

Erectile dysfunction can have a major impact on self-esteem and sexual relationships, greatly impacting a patient’s and their partner’s quality of life.

Viagra (sildenafil citrate) may help with erectile dysfunction, but can lead to or worsen orthostatic hypotension. Patients using a sildenafil treatment should avoid standing and walking for several hours after taking the medication. Injections of medication into the penis (intracavernous injections), which work to open its blood vessels, or the use of a vacuum constriction device also might be useful in treating erectile dysfunction.

Last updated: Jan. 7, 2021

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FAP News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.