Pediatric Lung Transplantation | For Parents

Medications

It is important to give all medications as prescribed. Please ask your transplant doctor or nurse if you have any questions about the medications. Remember the medication schedule has been tailored specifically for your child. Because the medications may interact with other drugs, check with your transplant doctor or nurse before taking any over the counter medications. In addition, be sure to tell your dentist or any other health care professional who treats your child about the about the medications he is taking. Tell your child "Even when you feel okay, you must still take your medications. They will be necessary for the rest of your life." We believe strongly that school aged children and adolescents need to be well-informed about and to take responsibility for their medications. Find a system of reminders or check offs that help your child and family towards lifelong regularity in taking these medications.

• Medications have brand and generic names. It will be important to become familiar with both the brand and generic names of the medications.
• Always carry the medications with you when you travel in case you are delayed or your luggage is lost.

Immunosuppressive Agents

These agents dampen your child’s immune system and lessens the chance of rejecting the transplanted lungs. The unfortunate trade-off is that your child will be more susceptible to infection. These medications must be taken on a strict schedule. Blood levels, white blood cell counts, and other laboratory tests will be monitored to check your child’s response to medications and to monitor side effects.

Cyclosporine(Neoral and Sandimmune)

Cyclosporin is the generic name, and Neoral and Sandimmune are the brand names. Cyclosporine is a drug which suppresses the T-Lymphocyte cell. Your child will be on cyclosporine for the rest of his life. Regular habits may be life saving!! It is important that your child take their cyclosporine at the same time every day. Usually 8 a.m. and 8 p.m. or 7 a.m. and 7 p.m. Pick a time that works best for your schedule. Cyclosporine comes in 25mg or 100mg capsules and in liquid form. The liquid form comes with a syringe. The liquid form of cyclosporine can be diluted before administration. Many patients find that chocolate milk works well, but just about any liquid is acceptable. Mix the cyclosporine in the solution of your child’s choice, and then have them take the medicine. Then add more liquid to the glass swishing it around to get any cyclosporine that may be left. Have your child drink the rinse solution also. Cyclosporine will stick to plastic so you must not use plastic or styrofoam. Glass is best. Most patients have found that teaching their children to take cyclosporine straight from the syringe is the best way to achieve consistent cyclosporine levels.

Cyclosporine dosing is based on plasma trough levels. A trough level is obtained by drawing a blood sample right before the cycolsporine is due (usually done before the morning dose). Do Not Give Cyclosporine Until After The Blood Sample Is Obtained. Our initial target trough level is 300 to 400 mg/ml. Later we may lower the target level. Initially CSA levels may vary often as your child’s body adjusts to this medicine.

Cyclosporine has important side effects, but many are dose-related and reversible. The ones we see most often are:

If a dose is ever missed, do not double the next dose. Call the transplant nurse or physician.

Cyclosporine pills are sensitive to extreme heat and may be altered by it. Check to see that your pills have not lost their shape or pliability before taking them.

It is important not to switch from Neoral to Sandimmune or vice versa. We prefer Neoral.

The following is a list of antibiotics which may compete with cyclosporin and may dangerously increase blood levels. If these drugs are necessary, your physician should call us so that the cyclosporin dose can be adjusted.

• ERYTHROMYCIN
• CIPROFLOXIN(CIPRO)
• CLARITHROMYCIN(BIAXIN)
• AZITHROMYCIN
• KETOCONAZOLE
• ITRACONAZOLE
• FLUCONAZOLE(DIFLUCAN)
• RIFAMPIN-markedly lowers CSA level

Azathioprine (Imuran)

Imuran is a drug which also suppresses white blood cell function more generally than Cyclosporine. Like all medication, Imuran has side effects. The most common side effect is a drop in the number of white blood cells. White blood cells fight infection. If your child has dangerously low white blood cells in their body, they may be more susceptible to infections, colds, or other viruses. This does not mean that they cannot fight infection. It means that their ability to fight off or resist infection is less than before your child was on Imuran. Imuran is available as a tablet or in liquid form and is usually well tolerated if taken with food or a glass of milk. The liquid form must be refrigerated and is stable for one month. The white blood cell count will be monitored and medication will be modified accordingly.

Possible side effects:

Mycophenolate Mofetil (Cellcept)

This medication may be used instead if Imuran at the discretion of the physician. This agent is an immunosuppressant and selectively suppresses T and B-lymphocytes.

How to take Cellcept: It is available only in oral form. It should be taken on an empty stomach and must be given at least 2 hours apart from magnesium supplement. Do not open the capsules.

Possible side effects:

Prednisone(Deltasone)

Prednisone is a member of the family of medications called Corticosteroids, which should be differentiated from anabolic steroids which some athletes abuse. Prednisone suppresses the body’s natural tendency to fight infection or foreign tissue, such as a transplant through both a broad anti-inflammatory effect and a strong general effect on lymphocytes. Although your child should never stop taking any of the anti-rejection medications, stopping prednisone suddenly can cause serious problems.

How to take prednisone: Prednisone is available in varying strengths and forms. Your child will take this medication with food usually in the morning. If stomach upset, burning, or pain develops, check with your transplant nurse or doctor. An antacid may be prescribed.

Prednisone has many possible side effects, and most are dose related:

Cortisteroids have another important role in immunosuppression. Episodes of acute rejections are treated with high dose intravenous cortisteroids (Solumedrol) for three days. Acute side effects of greatest concern are hypertension and high blood sugars in susceptible individuals (usually those with CF).

BLOOD PRESSURE LOWERING (Anti-Hypertensive medications)

Nifedipine( Procardia XL)

Captopril(Capoten)

These medications lower blood pressure and are often used in combination with other drugs. Remember, high blood pressure is a common side-effect of CSA.

Medications can lower blood pressure in many ways. Some dilate (open wider) the blood vessels. Others change the kidney’s effect to control blood pressure. Still others block part of the nervous system that increases blood pressure. Rather than include information on all of these medications here, your nurse, physician, or pharmacist will give you information on your specific medication when it is prescribed for you. It is important that you check your child’s blood pressure daily, and that they take any anti-Hypertensive medication exactly as instructed. We will take your blood pressure at each clinic visit. Blood pressure should also be taken if dizziness or severe headaches occur.

Nifedipine (Procardia XL)

Nifedipine is a sustained release capsule that cannot be chewed or crushed. Avoid fruit and fruit juices 30 minutes before and 1 hour after administration. Side effects include swelling, (usually of the feet) pounding or racing of the heart, nausea, rash, flushing of the skin, headache, dizziness, and fatigue.

Watch for symptoms of hypotension (low blood pressure): lightheaded, cold or clammy feelings, or feeling weak. Check your child’s blood pressure should these symptoms occur.

Diuretics or Water Pills

Furosemide(Lasix)

A diuretic pill is a medication that helps your body get rid of excess fluid by increasing urine production. Unfortunately, patients may urinate more frequently during the night. Your child may have been taking one of these medications before transplant. The diuretic may help control blood pressure or may offset the action of some medications, such as prednisone, that may cause you to retain excess fluid. It is important to have your child’s blood pressure and blood electrolyte (salt and potassium) levels checked frequently when taking diuretics (we will do this with each clinic visit). Electrolytes are necessary for the body to work properly. Symptoms such as muscle aches, nausea, headache, and fatigue may indicate that electrolyte levels are abnormal. You should report any of these symptoms to the transplant nurses or doctor.

A potassium supplement may be needed when your child is on Lasix. We will monitor potassium levels.

Prophylactic Medications

These are medications that your child will take to prevent infections or other complications.

Gancyclovir(Cytovene)

An antiviral drug against Cytomegalovirus that comes in oral and IV (intravenous) forms.

Gancyclovir is used to prevent or treat CMV disease in transplant recipients. If your child test positive for CMV prior to transplant (or if the donor test positive), a six week course of IV Gancyclovir will be indicated.

How to take Gancyclovir: IV Gancyclovir will be supplied by your homecare company, and a nurse will come to your home to instruct you on the infusion. Oral Gancyclovir may be tolerated better if taken with food.

Gancyclovir has a few possible side effects:

Nystatin (Mycostatin, Mycolex troche or lozenge)

An anti-fungal medication used to prevent or treat fungal infections such as thrush in the mouth or vaginal yeast infections.

How to take Nystatin: Nystatin can be taken as a liquid or a lozenge. It works as it contacts the mucous membrane. Unlike most oral medicines, it is not absorbed into the body and distributed via the bloodstream. The liquid should be swished around the entire mouth for a minimum of thirty seconds and then swallow. The lozenge is held in the mouth and allowed to dissolve completely. It may take thirty minutes to completely dissolve . Do not chew or swallow a lozenge whole. Take your Nystatin last (after your other pills) or after brushing teeth, and do not eat or drink anything for 15 to 30 minutes after taking it, otherwise you will wash away it’s effect. Nystatin vaginal cream or tablets are used if a female patient gets a vaginal yeast infection.

Possible side effects (rare):

• Stomach cramping or diarrhea.

Itraconazole or Ketoconazole (Nizoral)

An anti-fungal medications which is used to treat or prevent fungal infections. It also has the added effect of increasing cyclosporine blood levels.

How to take Itraconazole or Ketoconazole: You should take the medication daily as prescribed at about the same time each day. This should be taken with food to avoid stomach pains or cramping,. You should not take this with either an antacid or Zantac since this lowers the effect of the medication. Because it increases the cyclosporine levels, it is as important as taking your cyclosporine capsules.

Possible side effects:

Acyclovir (Zovirax)

Acyclovir is an anti-viral drug available as a cream, pill and in intravenous form, that treats or prevents viruses of the Herpes family. Your child may have been exposed to several of these viruses in their lifetime. Because your child must take anti-rejection medications after transplant, they are more susceptible to a reactivation of these viruses. Acyclovir is often used after transplantation to treat a herpes infection (cold sores or genital herpes), chicken pox, varicella zoster (shingles), and Epstein-Barr virus (mononucleosis).

How to take Acyclovir: If prescribed in tablet form, it may be helpful to take Acyclovir with meals to reduce stomach upset.

Possible side effects:

Septra(Bactrim, Co-Trimethoprim, or Sulfamethoxazole)

An antibiotic medication. Your child will be taking this medication to prevent a particular infection, called pneumocystis carini pneumonia.

How to take Septra: Your child will take Bactrim or Septra as "DS" which is "double strength" or "SS" which is "single strength", three times per week (Monday, Wednesday, and Friday). It should be taken with a full glass of water.

Some side effects include: low white blood cell or low red blood cells, nausea, vomiting, diarrhea, rash, itching, and increased creatinine.

Your child should stay out of direct sunlight or use sunblock/sunscreen when taking this medication. Exposure to sunlight without sunscreen even for brief periods of time may cause sunburn.

Ranitidine (Zantac) or Famotidine (Pepcid)

A strong blocker of stomach acid production. Most patients take one of these medications in the first weeks after transplantation to reduce stomach irritation.

How to take Zantac/Pepcid: Available in liquid, tablet or in intravenous form. Your child will take this once or twice daily depending on their symptoms.

Possible side effects:

• Constipation
• Diarrhea
• Nausea/Vomiting
• Decreased blood counts

MVI (Multivitamin)

A multivitamin is given to make sure your child gets all the proper nutrients to assist healing after surgery. Any type of multivitamin is fine.

Magnesium Supplement

Cyclosporine depletes the body of magnesium, therefore your child will be required to take a magnesium supplement, for most patients as long as they take their CSA.

Cisapride(Propulsid)

Aids in stomach motility. Poor stomach emptying is a common problem in many patients in the days and weeks after transplantation.