Wednesday, June 2, 2010

Cerebral Palsy

1. A mother brings her 13 year old daughter with cerebral palsy in to see you because she is concerned that her daughter has not started her menstrual cycle yet. You tell her the following:

a. We will start her on oral estrogen therapy because I am concerned about her delayed puberty

b. Research has shown that patients with cerebral palsy often have delayed menarche, with the median age of menarche at 14. If she has not started her cycle by 14, please return for re-evaluation.

c. We will start her on a gonadotropin releasing hormone agonist (GNRH).

d. We will start her on continuous oral contraceptives to manage her menstrual cycle.

(B is the correct answer. Since many patients with CP have delayed menarche, this may be normal for this patient, so estrogen therapy is not warranted at this time. C is incorrect because GNRH is a treatment option for girls with early puberty, not late. D is incorrect because she hasn’t had a menstrual cycle so she doesn’t need contraception yet. A good article to read for further clarification is the one by Zacharin.)

2. A couple comes to see you for preconception counseling. The wife has cerebral palsy and the couple wants to know what is the likelihood of passing cerebral palsy on to their child. You respond with the following:

a. Cerebral palsy is an autosomal dominant condition and each of your children have a 50% chance of inheriting it.

b. Cerebral palsy is curable, so let’s work on getting you better first and then we can worry about you getting pregnant.

c. Cerebral palsy is caused by an insult to the brain usually occurring during fetal development or infancy. We are not always certain what causes the insult to the brain but we know that it is not passed on like a genetic condition.

d. Due to your disability, I do not recommend that you get pregnant. Let’s talk about some contraceptive methods and possibly adoption.

(C is the correct answer. A is incorrect because cerebral palsy is not a genetic condition, therefore it cannot be autosomal dominant. B is incorrect because CP is a chronic condition that is not curable. The goal of treatment is to get the patient to their maximum level of independence. D is incorrect because CP does not prevent someone from getting pregnant and many CP patients have delivered babies with no complications.)

3. Sandy is a 23 year old female with cerebral palsy. She is confined to her wheelchair and has spasticity of arms and hands but has no cognitive impairment. She is obese. She lives alone with the help of a twice daily nurse visit. She works outside of her home so menses control is important to her. She would like to discuss contraception options with you. Which of the following would be the best option for her?

a. Depo-Provera shot every three months

b. Combined oral contraceptive pills

c. Transdermal patch

d. Levonorgestrel IUD

(D is the correct answer. The IUD is a long-term, reversible option that may induce amenorrhea for Sandy, which is something she desires. It also does not contain estrogen, so there is not an increased risk for DVTs like with the combined OCPs. Also, remember to teach Sandy about the signs and symptoms of an infection after IUD placement. A is not the best option because Depo has been shown to increase loss of bone density and since Sandy is immobile she is already at increased risk for that. B is not the best because of the increased risk for DVTs with estrogen containing contraception. C is not the best because the patch is not as effective in obese women.)

4. Amy is 20 years old and has cerebral palsy. She is here today for preconceptual counseling. During your history and physical, you learn that Amy also has epilepsy and is on Phenytoin. Which of the following drugs might you consider switching Amy to?

a. Lamotrigine

b. Valproate

c. Carbamezipine

d. Phenobarbital

(A is correct. Lamotrigine is the only class C anticonvulsant drug in this list. All the other drugs listed (B, C, and D) are class D drugs. Lamotrigine is the most widely used second generation antiepileptic and is a potential option for pregnant women with seizure disorders. Lamotrigine has anti-folate properties, so folate supplementation 1-4 mg is recommended. Obviously, a class C drug is not great, but remember that a seizing mother is really bad too.)

5. You have a 20 week G1P0 with cerebral palsy in your office. She has limited mobility and uses a walker for ambulation. Which of the following complications in pregnancy would you be least likely to see in this patient?

a. Deep vein thrombosis

b. Urinary tract infection

c. Fracture from falls

d. Autonomic dysreflexia

(D is the correct answer. Autonomic dysreflexia is an over activation of the sympathetic nervous system that occurs in patients with spinal cord injuries. It can be triggered in spinal cord injury patients during pregnancy. Answers A, B, and C are all things that you would be concerned about for your CP patient. Providers should be sure to give this patient information to prevent these complications along with information about the signs and symptoms of them. A great article to read about complications in pregnancy for women with disabilities is the one by Smeltzer.)

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