RAYNAUD’S PHENOMENON – Definition, Types, Causes, Risk Factors, Pathophysiology, Signs and Symptoms, Diagnostic Evaluation, Treatment and Management
DEFINITION
Raynaud’s phenomenon is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other areas. This condition may also cause nails to become brittle with longitudinal ridges. Due to vasospasm, decrease blood supply to the respective regions. Stress and cold are classic triggers of the phenomenon
TYPES OF RAYNAUD’S DISEASE
There are two types of Raynaud’s disease – Primary and Secondary:
- Primary Raynaud’s disease: in these cases, the cause of the condition is unknown. It does run in families, however, so a genetic cause is suspected. Primary Raynaud’s disease is five times more common in women than it is in men, and usually starts between the ages of 20 and 45 years.
- Secondary Raynaud’s disease: secondary Raynaud’s disease is so-called because it occurs secondary to another condition or factor, such as:
Medications that narrow the blood vessels, e.g. beta blockers
Hormone imbalances, e.g. hypothyroidism
Injury, e.g. frostbite
Occupational exposure to constant vibration (e.g. chainsaws) or repetitive movement (e.g. typing)
CAUSES
- Primary Raynaud’s (disease): Raynaud’s disease, or ‘Primary Raynaud’s’, is diagnosed if the symptoms are idiopathic. It often develops in young women in their teens and early adulthood. Primary Raynaud’s is hereditary.
- Secondary Raynaud’s (syndrome): Raynaud’s syndrome, or ‘Secondary Raynaud’s’, occurs secondary to a wide variety of other conditions. Secondary Raynaud’s has a number of associations:
- Connective tissue disorders:
Scleroderma
Systemic lupus erythematosus
Rheumatoid arthritis
Polymyositis
Mixed connective tissue disease
Ehlers-Danlos Syndrome
Eating disorders – anorexia nervosa
Obstructive disorders – atherosclerosis, Buerger’s disease, Takayasu’s arteritis, Subclavian aneurysms, Thoracic outlet syndrome
Drugs – betablockers, cytotoxic drugs-particularly chemotherapeutics and most especially bleomycin
- Cyclosporin
- Bromocriptine
- Ergotamine
- Sulfasalazine
- Anthrax vaccines whose primary ingredient is the anthrax protective antigen
Occupation
- Jobs involving vibration, particularly drilling, suffer from vibration white finger
- Exposure to vinyl chloride, mercury
- Exposure to cold
Others
- Physical trauma, such as that sustained in auto accident or other traumatic events
- Lyme disease
- Hypothyroidism
- Cryoglobulinemia
- Malignancy
- Reflex sympathetic dystrophy
- Carpal tunnel syndrome
- Magnesium deficiency
- Multiple sclerosis
- Erythromelalgia (the opposite of Raynaud’s with hot and warm extremities)
RISK FACTORS
Smoking worsens frequency and intensity of attacks, and there is a hormonal component. Caffeine also worsens the attacks. Sufferers are more likely to have migraine and angina than controls.
PATHOPHYSIOLOGY
Due to sudden vasoconstriction —- pooling of deoxygenated blood —- skin becomes bluish in coloration —- as a result of exaggerated reflow (hyperemia) due to vasodilatation —- a red color is produced when oxygenated blood returns to the digits after vasospasm spots —- there are characteristic changes in color described as white, red, blue, numbness, tingling, burning pain
SIGNS AND SYMPTOMS
- Pain
- Discoloration (paleness)
- Sensations of cold and/or numbness
- Swelling
- Tingling
- Raynaud’s also has occurred in breastfeeding mothers, causing nipples to turn white and become extremely painful
DIAGNOSTIC TESTS
- Digital artery pressure: pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mm Hg is diagnostic (positive)
- Doppler ultrasound: to assess blood flow
- Full blood count: this may reveal a normocytic anemia suggesting the anemia of chronic disease or renal failure
- Blood test for urea and electrolytes: this may reveal renal impairment
- Thyroid function tests: this may reveal hypothyroidism
- An autoantibody screen, tests for rheumatoid factor, erythrocyte sedimentation rate, and C-reactive protein, which may reveal specific causative illnesses or a generalized inflammatory process
- Nailfold vasculature: this can be examined under the microscope
TREATMENT (General Care)
- Environmental triggers should be avoided, e.g. cold, vibration, etc.
- Emotional stress is another recognized trigger
- Extremities should be kept warm
- Consumption of caffeine and other stimulants and vasoconstrictors must be prevented
Emergency Measures
- Keeping warm and maintaining a constant body temperature
- Wearing gloves and warm socks when out in the cold
- Not smoking – nicotine can narrow the blood vessels
- Not directly handling cold things, e.g. bottles of milk, items just out of the freezer
- Keeping the skin supple by using moisturizers
- Learning how to manage stress and emotional situations
- Avoiding medications that can aggravate blood vessel spasm, e.g. some cold and flu medications
- Medications to widen the blood vessels and promote circulation (calcium channel blockers)
- Medications to thin the blood, e.g. aspirin
- Treatment of underlying conditions in cases of secondary Raynaud’s disease
- Alternative therapies, e.g. massage, acupuncture
Drug Therapy
- Calcium channel blockers: (Nifedipine) or diltiazem
- Side effects: headache, flushing, and ankle edema; but these are not typically of sufficient severity to require cessation of treatment
- Angiotensin II receptor antagonists: (Losartan) reduce frequency and severity of attacks
- Vasodilator therapy: sildenafil (Viagra) improves both microcirculation and symptoms in patients with secondary Raynaud’s phenomenon
- Selective serotonin reuptake inhibitor: fluoxetine, a selective serotonin reuptake inhibitor
- Antidepressant medications: may reduce the frequency and severity of episodes if caused mainly by psychological stress
Surgical Intervention
- Sympathectomy: procedure can be performed. The nerves that signal the blood vessels of the fingertips to constrict are surgically cut
Nursing Management
Nursing diagnosis: risk for hemorrhage related to graft procedure
Goal: to reduce risk of bleeding
Interventions
- Monitor pulse rate
- Monitor central venous pressure
- Provide sterile dressing on wound
- Give vitamin K as per doctor’s advice
Nursing Diagnosis: pain related to disease condition as evidences by verbal communication
Goal: pain is reduced or lost
Intervention
- Assess for the presence of pain, the scale and intensity of pain
- Teach the client about pain management and relaxation with distraction
- Secure the chest tube to restrict movement and avoid irritation
- Assess pain reduction measures
- Provide analgesics as indicated
Nursing Diagnosis: risk for impaired gas exchange related to cough and pain for incision
Goal: to clear secretions from airway
Interventions
- Airway Management:
Open the airway with headtilt, chinlift, jaw thrust
Set the position to maximize ventilation
Use tools airway
Perform chest physiotherapy
Teach breathing deeply and coughing effectively
Perform suction
Auscultation of breath sounds
Give bronchodilators (collaboration)
- Oxygenation Therapy:
Provide humidification system of oxygen equipment
Monitor the flow of oxygen and the amount given
Monitor signs of oxygen toxicity