The MOUSE Care Plan
Medicate, Observer, Understand, Self-help, Early delivery
Ursodeoxycholic Acid - UDCA (Actigall, Urso)
UDCA is one of the most important treatments for ICP. It's natural bile acid composition improves maternal liver funtion by replacing the more toxic bile acids in bloodstream. Studies have shown that it also improves bile acid transport across the placenta, which may greatly reduce the risk of stillbirth associated with ICP.
Vitamin K therapy is a suggested course of treatment for all ICP patients. Choleastasis reduces the absorption of fat-soluble vitamins which can lead to Vitamin K deficiency, possibly resulting in serious complications including hemmorhaging in the mother and intra-cranial hemmorhaging for the infant. Oral, water-soluble Vitamin K supplements are recommended for the mother and upon delivery, the newborn should recieve an injection of Vitamin K. Breast milk contains little to no amounts of Vitamin K and therefore should not be relied upon for treatment purposes.
Raised bile acids increase the risk of premature labor. This threat coupled with the fact that all ICP babies should be delivered by 36 weeks often prompts the usage of steroids to increase the probability of lung maturity at birth. Research is strong that steroids given before 32 weeks gestation have a profound impact on fetal lung maturity. In addition to improving fetal lung maturation, there is some indication that steriods may also curtail some of the itching associated with ICP by suppressing hormone production.
Cholestyramine (Questran, Colestipol)
In previous years, cholestyramine and other cholesterol lowering agents were the front-line medications for treating ICP. As more information and studies have become available, cholestyramine has been excluded by many doctors for ICP treatment due to it's lack of effectiveness in aggressively lowering bile acids and it's potentially dangerous side affects. Cholesterol lowering agents are known to also inhibit certain fat-soluble vitamins, including Vitamin K, which is essential for blood clotting. Due to the fact that ICP patients are aleady at risk for Vitamin K deficiency, prescribing cholestyramine should be avoided to prevent maternal hemmorrhage or intracranial hemmorhaging in the unborn child.
Serum Bile Acid Testing
The most sensitive indicator of ICP is a rise of serum bile acid levels (amounts above 12-14 umo/l). Therefore, the a Serum Bile Acid test should be used to determine a diagnosis of ICP and bile acids tests should be repeated at least weekly until delivery and again afterwards in order to rule out underlying liver problems. Bile acids, whether acting directly or indirectly, trigger the intense itching associated with ICP and are believed to be the catalyst. The increase of serum bile acids in combination with itching is highly suggestive of a diagnosis of ICP. Any pregnant woman with raised serum bile acids needs to be treated to reduce the bile acids even if the ICP is thought to be the result of an underlying disorder.
Liver Function Testing
A Liver Function Test that measures the levels of transaminases (AST, ALT & ALK) in the bloodsteam should also be carried out weekly or twice weekly. It is important to note that liver enzymes elevate in the bloodstream as a result of elevated serum bile acids, and therefore a normal result of a Liver Function Test means nothing when concluding a diagnosis of ICP. It is possible for an ICP patient with raised Serum Bile Acid levels to have perfectly normal Liver Function Test results and still be at risk.
CTG, Doppler & Ultrasound Surveillance
Although the above mentioned surveillance techniques will not totally elimiate the risk of stillbirth, aggressive surveillance is suggested. Most ICP experts suggest some or all of the following fetal monitoring techniques to be administered twice weekly upon diagnosis or start of the illness and be repeated until delivery:
- Biophysical Profile - test that uses the nonstress test and ultrasound to examine fetal movements, heart rate, and amniotic fluid amounts.
- Cardiotocography - evaluates fetal heart rate.
- Doppler Flow Study - A type of ultrasound which use sound waves to measure blood flow.
- Ultrasound - diagnostic imaging technique which uses high-frequency sound waves to create an image of the baby's body and organs and the surrounding tissues.
Prothrombin Time (PT)
Due to the increased risk of Vitamin K malabsorption, clotting times (PT) should be checked at least weekly close to the date of delivery and at delivery to prevent the risk of maternal hemorrhage and intra-cranial hemorrhage in the infant. Intra-cranial hemorrhaging is a risk to the infant in utero as well as after delivery, so it is vital that the newborn infant receive a Vitamin K injection upon delivery.
ICP is believed to be a condition caused by a gene mutation resulting in the liver's inability to cope with high levels of estrogen present in the body. However, ICP is sometimes a result of an underlying liver disorder or other complication. It is normal for patients to continue itching for some time after delivery, particularly with cases that have been prolonged or in cases where a patient has had several pregnancies in a short time frame, but special attention should be given to patients whose itching intensifies after delivery or does not go away at all. Post-partum Serum Bile Acid testing and Liver Function testing are suggested for ICP patients to ensure overall health. A differential diagnosis may include Primary Bilary Cirrhosis, Hepatitis, AIDS, anemia, Benign Recurrent Intra-hepatic Cholestasis, and bile duct obstruction.
UNDERSTAND THE RISKS
Elevated levels of serum bile acids have been proven to increase the incidence of meconium passage, therefore increasing the risk of sudden stillbirth. Meconium passage is also associated with umbilical vein constriction which can lead to a reduction in umbilical flow. Meconium staining is reported often among ICP patients and one of the most dangerous risks associated with ICP. Proper medication and early delivery can help to eliminate this possible risk.
Fetal & Maternal Hemmorhaging
Cholestasis patients have a reduced ability to absorb fat-soluble vitamins (A,D and K). This may lead to Vitamin K deficiency. Because of the risk of intra-cranial hemorrhage in the infant and post-partum maternal bleeding, water soluble Vitamin K should be administered to both mother and baby. There have been reports of maternal hemorrhage as well as stillbirth in utero and post-partum due to ICP induced Vitamin K Deficiency.
ICP has been associated with a substantial rate of preterm birth. In a French study involving 40 ICP patients, 32.5% of the patients were reported to have preterm labor. Earlier presentations of ICP seem to carry an even greater risk of preterm labor, as well as twin or triplet pregnancies.
Lower Fat Intake
Many women notice that eating a lower fat diet helps with the severity of the itching they experience. This may be due to the fact that eating fats prompts the body to secrete bile acids into the stomach to begin the digestion process, which may mean more bile acids eventually entering the blood stream.
Water, Water & More Water
It is important that cholestasis sufferers have a good intake of water. Water helps to flush toxins out of the body and helps the body systems work more efficiently.
Milk Thistle, Dandellion Root, and SAMe
Milk Thistle and Dandelion Root or Tea are safe herbs for pregnancy are have been proven to greatly strengthen and benefit the liver. SAMe is a manufactured susbstance produced that has shown to have some benefits to ICP sufferers. In a study comparing SAMEe with UDCA, SAMe was found effective, but not as effective as UDCA. Before taking any herb or drug, please consult with your doctor or midwife. Black Kohosh is an herb known for it's benefit to the liver, but it contribute to preterm labor and should therefore be avoided before 36 weeks.
Low Stress Levels
Many women have noticed that high stress situations increase their itching. It helps to maintain as low of a stress level as possible.
Eliminate External Irritants
Although ICP itching is caused by internal irritants, it certainly doesn't help to have dry, irritated skin. The following is a list of suggestions to reduce external irritants to the skin: Keep skin moisturized, keep legs shaved and smooth, keep environment moist with a humidifier, wear cool & loose cotton clothing, keep temperatures low to keep blood flow slower, use ice-packs and cool baths for comfort, reduce bedding and linens at night, and use soft cotton slip covers on furniture when sitting.
Sarna lotion is a menthol lotion that many women find soothing. It does not reduce bile acids and is not a treatment, but rather a topical help for the itching. Many women find that rubbing Sarna and then sitting in front of a fan helps to cool and soothe the affected areas.
No Later Than 37 Weeks
ICP Specialists recommend that women with ICP, excluding diabetic mothers, be delivered at 36 weeks, no later than 37 weeks to prevent the risks that are associated with ICP. This protocol is recommended in controlled and mild cases alike. Bile acids can triple in value in a matter of 24 hours, and because there is no way to evaluate the bile acid level in the unborn baby, it is suggested to deliver early.
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