Here is a list of the drugs that Ashley takes every day to deal with her cystic fibrosis and the double-lug transplant she had more than two years ago.⁽¹⁾

Digestive

Along with causing lung function to degenerate over time, cystic fibrosis also causes several gastro-intestinal sequelae. As a result, medications commonly indicated for other pathologies are given to CF patients for the relief of various symptoms.

Ursodiol. 300mg, 1 capsule twice per day.

Ursodiol (brand name: Actigall) is typically a gallstone dissolution agent. It supplements the body’s production of bile acids salts, which are needed for the absorption of fats and various vitamins from the diet.

Pancrease. MT20, 3 capsules with each meal, 2 with snacks.

Pancrease is a brand name of Pancrelipase, an enzyme replacement therapy⁽²⁾ prescribed for patients with an improperly functioning pancreas. It aids in the digestion of starches, fats and proteins.⁽³⁾

Nexium. 20mg, 1 capsule once per day.

Nexium is a common brand-name version of Esomeprazole, a proton pump inhibitor,⁽⁴⁾ that causes the stomach to decease the amount of acid it produces.

Cystic Fibrosis-Related Diabetes Meds

Most people with CF also develop a specific type of diabetes. Like regular diabetics, they can be either Type I (they produce no insulin) or Type II (they don’t produce enough insulin).

Lantus. U-100. 12mg shot once per day.

Lantus is a brand name of insulin glargine, which is used to lower blood sugar in diabetic patients.

Novolin R. U-100. Taken only if blood sugar is very high.

Novolin is a brand name of Insulin Regular. It is also used to lower blood sugar, but in a much more fast-acting and dramatic fashion than Lantus.

Anti-Rejection

Prograf. 8.5mg in the morning, 9mg at night. (Varies according to blood levels.)

The active ingredient in Prograf is tacrolimus, a macrolide⁽⁵⁾ immunosuppressant. Tacrolimus suppresses some immunity of bodily fluids, but also – to a greater extent – cell-mediated reactions, such as rejection. Long-term use of tacrolimus increases the risk of cancer, serious infection, renal and hepatic failure.

A common side-effect (28% of patients) is dyspepsia, which is why Ashley wasn’t prescribed Nexium until after her transplant.

Prograf is available in 5mg, 1mg and .5mg capsules. Ashley must have one of each available to her at all times, and maintaining a back-up supply of at least one month is highly recommended by her doctors. In short, without Prograf she won’t be able to live very long.

Prograf is very expensive. A one-month supply of the 5mg capsules costs over $5,000. One of the few good points of the American health-care system (as of this writing, December, 2009) is that if a patient receives a transplant while covered by Medicare all prescriptions required as a result of the transplant are subsequently covered by Medicare for life.⁽⁶⁾

Mycophenolate. 250mg, 3 capsules twice per day.

Mycophenolate is the generic name for Cellcept. It extends the survival of allogenic transplants by inhibiting the proliferation of T- and B-cells and antibody production. (T-cells are part of the body’s cell-mediated immunity system, which is a response that doesn’t involve antibodies and complements. B-cells are part of the body’s humoral immunity system, which is mediated by secreted antibodies.)

Mycophenolate carries a strong risk to the human fetus. Women who are prescribed this are required to use two forms of contraception at all times.

One of the adverse effects of Mycophenolate is hypertension.

Prednisone. 5mg, 1 tablet once per day.

Prednisone is a corticosteriod⁽⁷⁾ that prevents the body from releasing chemicals⁽⁸⁾ that cause inflammation.

Blood Pressure

Hydrochlorothiazide.25mg, 1 tablet twice per day.

Hydrochlorothiazide lowers blood pressure and gets rids of extra salt and water in the body through the kidneys.

Metoprolol tartarate. 25mg, 1 tablet twice per day.

A lopressor, used to block chemicals that stimulate the body.

Amoldipine besylate. 5mg, 1 tablet once per day.

Amoldipine is the generic form of Norvasc, which relaxes the blood vessels and reduces blood pressure.

Hydralazine. 10mg. Only if hypertensive.

Also relaxes the blood vessels and reduces blood pressure, a direct-action smooth muscle relaxant.

Antibiotic

Bactrim. 160 mg, one capsule twice per day.

Bactrim is one of may brand names of sulfamethoxazole and trimethoprim, a medication use to treat a variety of bacterial infections.

Nebulized Aerosol Treatments

Albuterol Sulfate. 1 vial twice per day. (Every other month.)

Albuterol works at sites in the airways to relax the muscles and improve oxygen delivery. In Ashley’s case, Albuterol is not a medicine so much as a prep for Tobi.

Tobi. 1 vial twice per day. (Every other month.)

Tobi is a brand name of Tobramycin, an aerosol antibiotic used to fight infections.

Supplements and Vitamins

Iron. 325 mg. 1 pill twice per day. (More during menstration.)

Folic Acid. 1 tablet once per day.

Vitamin E. 400 IU. 1 pill twice per day.

Vitamin D plus Calcium. 600 mg. 1 pill once per day.

Other

In addition to Ashley’s Bag o’ Meds, we have several other apparatuses (apparati?) around the apartment that sometimes makes me thing we’re running some type of free clinic.

• Sphygmomanometer, or a blood-pressure machine.
• Glucometer, for measuring blood sugar.
• Peak flow meter. For measuring:

  • Peak expiatory flow (PEF),
  • Forced expiatory volume in one second (FEV1), and
  • Forced expiatory volume in six seconds (FEV6)

• And enough syringes and alcohol wipes to film six of seven Trainspotting squeals.

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