Health Info #8

Graft-versus-Host Disease (GVHD)
GVHD is a common complication following an allogeneic stem cell transplant. Twenty to fifty percent of patients undergoing a stem cell transplant with a related HLA-matched donor develop GVHD. The incidence is higher among older patients and those transplanted with stem cells from an unrelated donor or HLA mismatched donor.

GVHD is triggered by cells called T-cells. T-cells are a type of white blood cell that can recognize which cells belong in the individual's body,and which cells do not. When a donor's T-cells are transplanted into a patient, they perceive the patient's organs and tissues as foreign cells, and orchestrate an immune system attack to destroy them.

GVHD is often discusses as if it were a single disease. It is, in fact, two diseases: acute GVHD and chronic GVHD. Patients may develop one, both or neither. Acute and chronic GVHD differ in their symptoms, clinical signs and time of onset.

Acute GVHD
Patients who develop acute GVHD usually do so during the first three months after transplant. The earliest sign is often a faint rash on the patient's chest or back. A skin rash may also appear on the patient's hands and feet. The rash may spread to other parts of the body and develop into a general redness, similar to a sunburn, with peeling or blistering skin.

Acute GVHD can also affect the stomach and intestines causing cramping, nausea and watery or bloody diarrhea. Jaundice may indicate that acute GVHD has affected the liver.

Physicians grade the severity of acute GVHD according to the number of organs involved and the degree to which they're affected. Acute GVHD may be mild, moderate or sever or life-threatening.

Prevention and Treatment of Acute GVHD
To reduce the risk of developing acute GVHD, patients are typically given a combo of cyclosporine and methotrexate, tacrolimus and methotrexate or cyclospring and mycophenolate mofetil.

Stages of acute GVHD
Stage 1 (mild); a skin rash over less than 25% of the body.
Stage2 (moderate): a skin rash over more than 25% of the body accompanied by mild liver, stomach and intestinal disorders.
Stage 3 (severe): redness of the skin, similar to a sunburn and moderate liver, stomach and intestinal problems.
Stage 4 (live-threatening): blistering, peeling skin and severe liver, stomach and intestinal problems.

Chronic GVHD
Chronic GVHD develops during or after the third month post-transplant. Patients with chronic GVHD usually experience skin problems that may include a dry itching rash, a change in skin color, and tautness or tightening of the skin. Liver abnormalities, dry or burning eyes, dry mouth, mouth sores, infections and stomach irritations are also common symptoms of chronic GVHD

Less frequently, patients experience skin scarring, partial hair loss, or premature graying of the hair. Others develop severe liver problems, vision difficulties, heartburn, stomach pain, difficutly swallowing, weight loss or breathing difficulties. Lung problems are a common complication of chronic GVHD. These can be serious and require prompt treatment.

patients who have had acute GVHD have the greatest risk of developing chronic GVHD. Older patients, those transplanted with stem cells from an unrelated or HLA mismatched donor, and those transplanted with stem cells that were collected from the bloodstream rather than with bone marrow may also have a greater risk of developing chronic GVHD.

Symptoms/side effects of chronic GVHD
Most common- rash, itching, general redness of skin, dark spots, tautness of skin, jaundice, abnormal liver tests, dry,burning eyes, dryness or sores in the mouth, burning sensation when eating acidic food, bacterial infection.
Less common- skin scarring, partial hair loss, premature graying, severe liver disease, vision impairment, heartburn, stomach pain, difficulty swallowing, weight loss, contractures, difficulty breathing, bronchitis, pneumonia.

Health Info #7

Preparative Regimen
The preparative regimen (also called the conditioning regimen) is the high-dose chemo and/or radiation administered to the patient during the week preceding their transplant. The preparatie regimen has 2 objectives: to destroy the patient's disease, and to suppress the patient's immune system so that the donor stem cells can engraft and begin producing healthy blood cells. Depending on the disease being treated, the preperative regimen may consist of one or more chemo drugs, or a combination of chemo and total body irradiation (TBI).

High-Dose Chemotherapy
Most preparative regimens include high-dose combination chemo. The chemo drugs are administered intravenously. If the chemo drug busulfan is part of the preparative regimen, it may be administered IV or taken in pill form by mouth. The exact combination and dosage of chemo drugs and radiation used in the preparative regimen varies according to the disease being treated and the preferred treatment plan of the transplant facility. Since researchers are constantly investigating promising new drug combinations and dosages, two tranplant centers may use different preparative regimens to treat patients who have the same disease.

Side Effects
HIgh-dose chemotherapy and TBI are toxic to normal tissues and organs, as well as diseased cells. Nausea, vomiting, diarrhea, mouth sores and temporary hair loss almost always occur to varying degrees regardless of which preparative regimen is used. Sever or long-term damage to organs and tissues occurs less frequently.

Nausea, Vomiting and Diarrhea
Nausea and vomiting are common following all preparative regimens, but can usually be controlled with medications. Drugs called antiemetics are used to treat nausea (emesis means vomiting thus antiemetics are drugs that prevent vomiting.

The feeling of nausea is controlled by the brain, not by the stomach. Many antiemetics act on the central nervous system to counteract this side effect. Antiemetics can cause additional side effects such as anxiety, drowsiness and restlessness. Occasionally, muscle tightness, uncontrolled eye movement and shakiness can occure. These drugs reactions can be frightening, but are usually less serious than they appear. Lowering the dosage of the antiemetic, or administering an antihistamine usually reduces or eliminates these side affects.
Diarrhea following the preparative regimen is also common. Anti-diarrhea drugs sedate the nerves in the gastrointestinal area, slowing down muscle contractions and the diarrhea.

Mouth, Throat, Skin and Hair
High-dose chemotherapy and radiation target rapidly dividing cancer cells. However, some normal cells such as those that line the mouth, throat and gut, as well as hair and skin cells, also divide rapidly. These cells can be temporarily damaged by high-dose chemo.
Mouth sores (mucasitis) and throat discomfort (stomatitis) typically appear four to eight days following the preparative regimen. Topical anesthetics or narcotis given IV such as morphine, are used to relieve this discomfort. Frequent brushing of the teeth and gums with a soft brush or sph9onge and rinsing with a solution of saline can be soothing and help prevent mouth infections.
Mucositis often makes eating difficult or impossible. Patients may be fed through IV until the discomfort subsides. IV feeding is also used if the stomach is unable to absorb sufficient nutrients (malabsorptions) as a result of temporary irratation cause by the preparative regimen. Antacid medications may be given to counteract stomach irritation.

Temporary hair loss (alopecia) always occurs following the preparative regimen. Hair loss changes a patient's appearance and for some can be very distressing. Some patients prefer to shave their heads or cut their hair very short before hair loss begins. Hair normally gows backr within three to six months following the transplant. Sometimes the amount of curl or thickness of the new hair will differ from the patient's hair pre-transplant. In rare cases, alopecia may be permanent.

Skin rash is common following preparative regimens that include TBI, busulfan, etopside, carmustin or thiotepa. At some centers showers are recommended on hour and six hours after infusion of thiotepa to reduce the likelihood of developing a rash.
Less often hyperpigmentation- dark spots on the skin- occurs. These usually fade over a period of one to two months.

Bladder Irritation
Bladder irritation, sometimes evidenced by bloody or painful urination, can occur following the preparative reigmen, particularly those that include cyclophosphamide or ifosfamide. Increasing the rates of IV fluids, using a Foley catheter to irrigate the bladder and administering a drug called Mesna are techniques commonly used to prevent or treat this problem.

Liver, lungs and Heart.
Temporary organ damage can occur following high-dose chemo. It is usually both mild and completely reversible.
Liver blood test abnormalities occur in 50 percent of patients following the preparative regimen, but only a small fraction will develop actual liver damage. Patients may experience jaudince (yellowing of the skin), significant weight gain due to fluid retention, and abnormal blood levels of liver enzymes and bilirubin (a pigment produced during the break-up of red blood cells). Resting the liver and avoiding medications that aggravate the condition are the usual treatments until the liver heals itself.

Breathing irregularities can also occur following the preparative regimen. Ten to twenty percent of patients develop non-infectious pneumonia during the first four weeks post-transplant. In most cases, injury to the lungs is mild and temporary, but some patients do experience long-term breathing problems.

Mild, temporary heartbeat irregularities (arrhythmia) or rapid heartbeat can occur following the preparative regimen, particularly those that include clyclophosphamide or carmustine. Severe or long-term heart problems are very rare.

Confusion
Confusion or altered thinking are occasional, temporary side effects of the preparative regimen, or of drugs used to control other side effects. Confusion and altered thinking can be frightening both to the patient and to loved ones who observe it. It helps to remember that these problems are temporary and reversible, and can often be managed by changing the dosage or type of drugs the patient is receiving.

Muscle Spasms and Cramping
Muscle spasms are a common problem post-transplant. They are sometimes caused by an imbalance in electrolytes- minerals found in the body such as potassium, magnesium and calcium. These minerals must be maintained at certain levels to prevent organ malfunction. Muscle spasms can often be resolved by taking potassium, calcium, magnesium or phosphate supplements orally. Ask your doctor to prescribe the supplement, since not all sources of these minerals are absorbed equally well by the body. If there is no electrolyte imbalance, vitamin E or quinine in pill form sometimes resolves or reduces the problem.

Reproductive Organs
Damage to reproductive organs from high dose chemo is common, and usually results in permanent infertility. Patient age, gender, stage of sexual maturity, and dosage of chemo all affect the likelihood of becoming infertile post-transplant. In addition, women often experience premature menopause.

Other Long-Term Side Effects
Premature cataracts occur in approximately 20 percent of patients who undergo TBI. Cataracts may also occur following treatment with high-dose busulfan. Cataracts can be surgically removed, usually in an outpatient setting.

Some patients experience numbness and tingling in their hands and feet, due to nerve damage caused by the preparative regimen and prior chemo. The damage is usually permmanent. However, in a few patients there has been slow re-growth of nerve tissue that eventually reduces numbness and tingling. The problem is seldom resolved completely.

Mild to moderate learning disabilities may occur in children who've had a stem cell transplant, especially if the preperative therapy includes TBI. Younger children often experience delayed growth as well. Hormone therapy may be recommended to promote growth if this problem occurs.

Children transplanted before the age of five may also experience significant dental problems such as loose teeth, tooth loss, dry mouth and inability to wear braces. It is important that they be followed by a dentist who is experienced in treating child who've undergone high-dose chemo and/or TBI.

She's a survivor

Alivia is at home recovering from her splenectomy. Thanks to all for the prayers and well wishes!
Pic courtesy Will Haughery.

Health Info #6

Treatment
Your child will receive 4 to 8 days of high dose chemotherapy, and maybe radiation, to destroy any remaining cancer or leukemia cells. This is called conditioning therapy. He/she will get a combination of drugs to get the most benefit from each with the fewest side effects. You can expect your child to have nausea, vomiting, diarrhea, hair loss and skin changes. We will give your child medications and take other measures to decrease the side effects whenever possible.

Your child will be given large amounts of IV fluids throughout the chemo. All of the IV fluids will be given through her central venous catheter. Each day your child will be seen and examined by several people, including a registered nurse, resident, and transplant doctor. All the staff caring for your child are highly skilled and specifically trained for stem cell transplantation.

Your child will be monitored closely throughout his/her stay. Vital signs will be taken at least every 4 hours, and weight will be checked early each morning. These things tell us important things about the care your child needs. A chest x-ray may be performed during your child's hospitalization. This is a routine test that will usually be performed in you child's room.

Lab work will be checked daily. The nurses will draw blood from your child's catheter early every morning.

Once chemotherapy has been completed, your child will become neutropenic. This means he/she has very few white blood cells and is at the most susceptible time to develop an infection. Your child may run a temperature and will be given antibiotics during this period to treat any infection. These antibiotics may need to be changed throughout the course of treatment. Your child's doctor or nurse will explain all of the treatments.
Throughout the hospital stay, and sometimes after discharge, your child will receive blood and platelet transfusion as needed to maintain his/her blood counts.

It usually takes about 2-3 weeks for your white blood cell count to start increasing. As this happens, your child will feel better and eventually be discharged.

Transplant day
Your child will be examined carefully by the doctor the morning of transplant. The stem cell product looks like blood, but thinner and redder. It will be given through the Hickman catheter. The staff will check your child's blood preassure, pulse and temperature frequently during the transplant. The infusion procedure may last anywhere from a few minutes to several hours. Since the stem cells are very similar to your child's own, he/she should not have any problems from it. But if he/she starts having fever, chills or any other unusual feelings, tell the nurse right away.

Since the autologous peripheral stem cells are frozen, a special water bath will be brought into the room to thaw the frozen cells. This first thing you and your child may notice is the taste and/or smell of garlic or creamed corn. This is the preservative used in the storing of the cells. A few people are allergic to this preservative. Your child's nurse will have medicaiton to give to prevent and to treat an allergic reaction.

After the Transplant
You and your child may feel somewhat "let-down" right after the transplant procedure has been completed. There were no fireworks and your child doesn't feel any different.

During the next several weeks we will wait for the stem cells to grow. The transplanted stem cells enter the marrow cavities inside your child's bones by way of the lungs and spleen. The cells begin to divide and produce red cells, white cells and platelets.
"Engraftment" is the term used when your child's bone marrow begins to make these normal cells.

Your child's white blood cell count will still be low because of the chemo so he/she is at risk of infection.

This is an anxious time. The isolations may get boring. You may begin to worry about how differnet things will be for you and your child when he/she is sent home. YOu may worry about what will happen if the transplant doesn't take. Your child's doctor can talk about other choices if that becomes necessary.

Health Info #5

Daily Routine
The skin, mouth and rectum are especially prone to irritation during transplant and will require special care for comfort and to prevent infection.

Skin Care
Everyone has germs on their skin that can cause an infection. Good skin care is an essential part of preventing infections. Your child will need to shower or wash every day using antibacterial soap. We can provide the soap or you may bring a brand of soap you prefer. Daily washing is very important even on days when you do not feel like it. Moisturizing lotion is helpful after the daily bath to prevent drying.

Mouth Care
You will need mouth care 4 times a day beginning at admission. Your nurse will go over the procedure with you. This procedure may chage and be done more often if severe mouth sores develop. When the mouth is healing well, it may be decreased. The nurses will check your mouth several times a day for signs of infection. Let them know of any pain or discomfort. Transplant patients should not drink hospital tap water or ice made from it. You will be given bottles of sterile water for drinking and mouth care.

Peri-Anal Care
Gentle, thorough cleaning needs to be done after every bowel movement. This is especially important if the child is having diarrhea or frequent bowel movements. Pat gently rather than wiping. Pat dry with a clean wipe. A barrier ointment should be used after each cleaning.

Bleeding Precautions
To prevent bleeding, you need to take some special precautions to protect your child. Adolescents should use electric razors instead of blade razors. Discourage child from blowing or picking nose. Keep fingernails trimmed or filed short. make sure your child wears slippers or shoes when out of bed.

Activity
Allow rest for 15-20 min between activites. Encourage him/her to move slowly from lying to sitting to standing so he/she wont' get dizzy. Encourage to stay as active as possible.

Happy Holidays!

Thanks to everyone who has continued to inquire about Livi's health, donated time or resources, and everyone who has prayed or sent well wishes to the family.
As we look forward to 2007, we hope that it will be a year for healing and happiness.
May you all have a blessed holiday season and we wish you all a successful, healthy and joyful new year!

Health Info #4

Peripheral Stem Cell Collection:
The process of removing stem cells from the bloodstream is called apheresis. Most patients who have autologous transplants will have stem cells collected from their peripheral bloodstream. Also, allogeneic donors may donate stem cells from their peripheral bloodstream.

The Pheresis Process:
Stem cells collection in small children is performed on the inpatient unit after a central venous pheresis catheter has been placed. Stem cell collection in older children and adults will be performed in the Blood Bank. The pheresis process takes about 4-6 hours. It may take more than one day of pheresis to collect enough stem cells for the transplant. Sterile tubing is connected from the pheresis catheter to the pheresis machine and back to the other side of the catheter. In larger donors without a catheter, the tubing will connect to an IV in each arm. The donor's blood will pass through the pheresis machine, which will separate and collect the stem cells and return the other blood cells to the donor. Allegeneic stem cells will usually be processed and infused the same day as the collection. Temporary side affects of apheresis include low calcium. low potassium, low platelet count and low red blood cell count. Transfusions and electrolyte infusions may be used to prevent or manage these side affects if they occur.

Health Info #3

Final Evaluation
If the tests and interviews show that a stem cell transplant will be helpful, the doctor will hold a conference with you. As part of the final evaluation you may have a consultation with a surgeon to discuss putting in a double-lumen Hickman Catheter. This is used to give medicines and blood products during and shortly after transplant.

Central Venous Catheters

Central Venous Catheters are hollow tubes made of a special soft plastic. They are used to draw blood and to give medicines and blood transfusions. The CVC is inserted in the operating room under general anesthesia (completely "asleep"). The day before the surgery, you will receive a call with instructions about when to come to the Same Day Surgery unit. You will also be given instructions as to when to stop eating and drinking.

The Procedure

When "asleep" from anesthesia, the surgeon makes a small incision near the collarbone and guides one end of the catheter into a large vein near the heart called the superior vena cava. The other end of the catheter is passed under the skin for a couple of inches and brought out through a small incision on the chest. This spot on the chest is called the exit site. The exit site will be covered with a bandage and you will be able to see several inches of the catheter outside of the bandage. The catheter will have two lumes or sides.

Before you are sent home, you will be given a prescription for pain medication since the catheter area will be sore for a few days. Keep the exit site dry while bathing or showering especially for the first 2-3 weeks after line placement

After catheter placement, there may be a small amount of bleeding at the exit site.

A donor may need to have a similar catheter placed to collect peripheral blood stem cells. This catheter will be placed the day before or the day of the first stem cell collection. It will be removed before the donor goes home.