The following excerpt is taken from Chapter
20 of Childhood Leukemia: A Guide for Families, Friends, and Caregivers, 2nd
Edition by Nancy Keene, copyright 1999 by O'Reilly & Associates, Inc.
For book orders/information, call (800) 998-9938. Permission is granted to print
and distribute this excerpt for noncommercial use as long as the above source is
included. The information in this article is meant to educate and should not be
used as an alternative for professional medical care.
Marrow is withdrawn
(harvested) from the large bones of the hips. While the donor or patient (for
autologous transplant) is under general anesthesia, the doctor inserts a large
needle into the bones of the hips and withdraws bone marrow. This procedure is
done up to fifty times to draw out a total of one to two pints. The entire
process takes less than one hour. After marrow donation, some hospitals keep the
donor overnight, while others discharge the same day if the donor is not in
pain.
Because the amount of
marrow that is removed contains less than five percent of the donor's developing
blood cells, it only takes a few days for the body to replace the marrow. The
donor or child is usually sore for a day or two, and may feel a bit tired for
several days. The recovery time varies from donor to donor.
After no match was found for Christie
in the 4,000 donors that we signed up and typed, I became her donor. We were
just a partial match. Being her donor was just wonderful. It was Mother's Day
weekend, and I was just full of faith and love. I thought this is it, God has
given me a second chance to give life to this child. I had a very powerful
feeling that it was so special that I could do this for my daughter. I really
felt that this was it, it was going to work.
It was
uncomfortable for a couple of days, and I was a little bruised. But the people
there were wonderful, and they really followed me closely. I'm still on the
registry; if someone called me tomorrow, I'd be on a plane. It's a very
rewarding feeling, a gift from God.
• • • •
•
Jody's
two-year-old brother Christoph was a perfect match. I stayed with him when he
donated marrow, and my husband stayed with Jody. Christoph seemed to handle the
marrow donation easily. Although he had some nausea in the recovery room, he was
up and running around late that afternoon saying, "I the donor." He
felt very proud. I knew he was somewhat sore because he said, "My diaper
hurts."
Prior to the actual
transplant, the patient's bone marrow is destroyed using high-dose chemotherapy
with or without radiation. This portion of treatment is called conditioning. The
purpose of the high-doses of chemotherapy and radiation is to kill all remaining
cancer cells in the body and make room in the bones for the new bone marrow or
stem cells.
Conditioning regimens
vary according to institution and protocol, and also depend on the medical
condition and history of the patient. Typically, the chemotherapy is given for
two to six days, and radiation (if part of conditioning) is given in multiple
small doses over several days.
They got JaNette into her second
remission in December, then gave her maintenance drugs to keep her there until
they were ready to start transplant conditioning in April. So, unlike some of
the other kids, she went to transplant healthy and strong. JaNette had 1200 rads
of total body radiation in five increments--two the first day, two the second
day, and one the third day. Then she had two days of incredibly strong
chemotherapy, one day of rest, then the transplant.
The transplant itself
consists of simply infusing the marrow or stem cells through a central venous
catheter or IV into the patient, just like a blood transfusion. The marrow or
stem cells travel through the blood vessels, eventually filling the empty spaces
in the long bones. Engraftment is when the new marrow begins to produce healthy
white cells, red cells, and platelets. This typically occurs from two to six
weeks after transplantation. Complete recovery of all components of immune
function can take from one to two years.
I couldn't believe how beautiful the
bone marrow was--a bag of shimmering red liquid. It just glistened. It meant
life.
• • •
• •
My dad donated marrow for my
transplant. He said he was sore and doing the "bone marrow hop," but
he made it to my room that day to see his marrow transfused into me. I really
felt different as I was getting the bone marrow, like I was getting so much more
energy.
• • •
• •
I cannot say enough good things about
the transplant center. They were very family-oriented, allowed us in the room 24
hours a day. I was allowed to sleep in bed with her ( I just told the nurses to
make sure to poke her and not me). The nurses were wonderful, and I still think
of them as family. We didn't get very close to the other families; we tended to
stay in our own child's room. One day a family would be there, then the next day
they would be gone. I got to the point where I just didn't want to know.
• • •
• •
The transplant went well except that he
was 50 days in the laminar flow room so that we couldn't touch him. Five years
old and we couldn't touch him. We had these gloves that we could use to reach
in. But Jody was such an accepting kid, he adjusted. We played a lot of cards
and at night we'd turn off the lights, and he'd imagine soaring out the tenth
floor window. He'd just fly out of that room. He was on hyperal (IV nutrition)
and lots of prednisone and other drugs, and he had a few tantrums. Once he
pulled out all of his needles and threw them against the wall. I thought that it
was a pretty healthy response, but the social worker showed up immediately.
• • •
• •
We were prepared to stay five months in
or near the transplant center, but we went home after only two and a half. She
had the normal nausea, so she was on TPN (total parenteral nutrition) from
transplant May 5 until the end of June. Until she could take her medications by
mouth, I did some IVs at home. She had her last platelet transfusion in July. It
took until the following June for her counts to normalize, and for her to start
producing T cells. We feel lucky that things went so well.
Donor and patient
confidentiality are closely guarded. The NMDP allows letter exchanges or
meetings if both donor and recipient express a strong desire to do so, but only
after a year has passed since the transplant.
Bone marrow
transplant takes a heavy emotional toll on the child, the parents, and the
siblings. It can be a physically and mentally grueling procedure, with the
possibility of months or years of aftereffects. Most transplant team members are
extensively trained to meet the needs of the patient and family during the
transplant and long convalescence. The team includes physicians and nurses, as
well as psychiatrists, social workers, chaplains, educators, nutritionists, and
child life therapists.
Christie had many painful complications
after the unmatched allogeneic transplant. I remember lying next to her and
saying, "Christie, if Mommy could take this away from you I would."
She looked at me and said, "Mommy, I love you so much, I would never want
you to go through this." But then she went on to say, "It's not so
bad, I met a lot of people, we got to travel, Daddy didn't have to work,
Danielle and Nicole got to spend time with Daddy." She just saw something
positive in everything, even this terrible disease.
Christie was
also very, very funny. She loved having me be the donor, and she started calling
me her "blood sister." She kept saying, "Ma, when am I going to
start being funny like you?" and I'd tell her that she was way beyond me.
Her humor was contagious.
• • •
• •
Leah was feeling good and was very
healthy when she went into the laminar air flow room. We lived near the
transplant center, and she had at least twenty visitors a day. I think the
visits and the nonstop telephone conversations really kept her spirits up. I
think the hardest part of the transplant was being in the laminar air flow room
(LAF). They gave me a sterilization wash, put me in some boots, and told me I
couldn't touch anything. I felt like a space person. Then I went into the room
for two months. I really missed touching people. I cried when I got out and
hugged my mom.
• • •
• •
What helped me the most was the
decorations and having a positive attitude. My mom decorated the area outside
the LAF room with balloons, cards, and posters. It was hard to take the
medicine, so my mom made a huge poster to mark off how well I did. Every time I
took my medicine, I got a sticker. When I got one hundred stickers, I got some
roller blades.
• • •
• •
I felt great during the transplant and
at the center, but when we came home it was very, very difficult. We had just
moved before my daughter was transplanted, so we didn't have a good support
system in place. She had been in the hospital, then transplant center for almost
a year, and then we had to stay at home for another year. For a few months she
would spike a fever every time someone who was not in the family came in the
house. So my young son could never go to friends to play or have friends over.
We just stayed home. It was very hard, and we all felt very isolated.
Often so much time
and energy is focused on the child who needs the transplant that the needs of
the sibling donor are overlooked. Siblings need careful preparation for what is
about to occur, and all questions need to be answered and concerns addressed.
Parents need to be careful that the sibling donor realizes that the results of
the BMT are out of everyone's control, and that the sibling is not responsible
for either her brother's life or death.
Some children have a
smooth journey through the transplant process, while others bounce from one
life-threatening complication to another. Some children live, and some children
die. There is no way to predict which children or teens will develop problems,
nor is there any way to anticipate whether the new development will be merely an
inconvenience or a catastrophe. This section will present some of the major
complications that can develop post-transplant, and the experiences of several
families in facing these problems.
Engraftment is when
the donated marrow takes up residence in the patient's bones and begins to
produce healthy blood cells. In cases where a child has received HLA-matched
marrow from a sibling, engraftment failure is less than 5 percent. In contrast,
children receiving marrow from partially HLA matched family members or unrelated
donors, graft rejection occurs in 5 to 25 percent of patients.
Most infections
following transplant come from organisms within the body (e.g., CMV, gut
bacteria). Good handwashing and adequately controlled ventilation can help
minimize infections with staph and fungi.
The immune system of
healthy children quickly destroys any foreign invaders; not so with children who
have undergone a bone marrow transplant. The diseased immune system of these
children has been destroyed by chemotherapy and radiation to allow the healthy
marrow or stem cells to grow. Until the new marrow or stem cells engraft and
begin to produce large numbers of white cells (two to six weeks), children
post-transplant are in danger of developing serious infections.
To prevent and combat
bacterial infections, children receive large doses of several kinds of
antibiotics if their temperature goes above 101°F (38.5°C) any time in the
first weeks after transplant. Ironically, the antibiotics used to treat bacteria
allow fungi to flourish. Fungal infections often follow prolonged neutropenia
and antibiotics and can't be prevented easily.
Leah had so many problems with
infections and getting her counts back up that she spent two years on steroids,
cyclosporin, and monthly gammaglobulin.
• • •
• •
During the related mismatched
allogeneic transplant, I developed no mouth sores, no infections, no GVHD. Just
a headache the whole time. I do miss being an athlete, I do miss the friends
that I lost, and I do miss my blond hair (it came back in brown, so I tried to
dye it blond and it turned bright, fluorescent red). But I can deal with those
things. To survive I think you need luck, a positive attitude, and a decorated
room to help you stay cheery.
After the first month
post-transplant, children are also susceptible to serious viral infections, most
commonly herpes simplex virus, cytomegalovirus, and varicella zoster virus,
particularly if they have GVHD. These can occur up to two years after the
transplant. Viral infections are notoriously hard to treat, so many centers use
prophylactic acyclovir or granciclovir or immunoglobulin to prevent these
infections.
CMV is usually
preventable if the patient and donor are both CMV-negative and all transfused
blood products are CMV-negative or filtered to remove white blood cells.
Interstitial
pneumonitis, a sometimes fatal form of pneumonia, is most common the second or
third month post-transplant. It is very uncommon after autologous transplants
and is most often associated with GVHD after allogeneic transplants.
Preventing infections
is the best policy for those children who have had a bone marrow or stem cell
transplant. The following are suggestions to minimize exposure to bacteria,
viruses, and fungi:
For more information
on infections, obtain the September 1993 BMT Newsletter,
"Infections," or read Bone Marrow Transplants: A Book of Basics for
Patients, by Susan Stewart.
Graft-versus-host
disease is a frequent complication of allogeneic bone marrow transplants. It
does not occur with autologous or syngeneic transplants. In GVHD, the bone
marrow or stem cells provided by the donor (graft) attack the tissues and organs
of the BMT child (host). Approximately 30 to 50 percent of persons who have a
related HLA-matched transplant develop some degree of GVHD. The incidence and
severity of GVHD are increased for those children who receive unrelated or
mismatched marrow, but are decreased if cells that cause GVHD are reduced prior
to infusion. The majority of GVHD cases are mild, although some can be
life-threatening.
There are two types
of GVHD: acute GVHD and chronic GVHD. Patients can develop one type, both types,
or neither. Acute GVHD usually occurs at the time of engraftment or shortly
thereafter. Donor cells identify the patient's cells as foreign, and may attack
the patient's skin, liver, stomach, or intestines. Allogeneic BMT patients are
given drugs before and after transplant in an attempt to prevent GVHD. Symptoms
of acute GVHD can range from mild skin rash to severe, sometimes fatal
infections, or liver, stomach, and intestinal problems. Acute GVHD is treated
with cyclosporin and steroids (prednisone, dexamethasone). Methotrexate may be
given to prevent GVHD.
JaNette's transplant was on May 5 and
her ANC was up to 1000 on May 30. That's when her graft-versus-host started. It
doesn't look like a regular rash, more like pinpoint red dots under the skin.
It's very itchy, and then it starts to peel. She looked like she had leprosy!
She had very little internal graft-versus-host disease. She's a year and a half
post-transplant, and she still broke out in a rash the last time they tried to
taper her off the cyclosporin.
• • •
• •
Ryan died from acute graft-versus-host
disease. It destroyed his liver. It was a hard death, and we felt that it robbed
us of whatever time he would have had left if he didn't have the BMT.
Chronic GVHD usually
develops after the third month post-transplant. It primarily affects the skin
(itchy rash, discoloration of the skin, tightening of the skin, hair loss), eyes
(dry, light-sensitive), mouth and esophagus (dry, tooth decay, difficulty
swallowing), intestines (diarrhea, cramping, weight loss), liver (jaundice),
lungs (shortness of breath, wheezing, coughing ), and joints (decreased
mobility). This list may seem overwhelming, but remember that only some patients
develop chronic GVHD, and those who do may experience all, a few, or only one of
these symptoms.
A year and a half after the transplant,
they tried to taper my daughter off the cyclosporin. Her liver function counts
went way up, and she broke out in a rash all over her body. The bottoms of her
feet were so blistered that she couldn't walk. She has permanent dark splotches
all over her torso, but the ones on her face and neck gradually faded away.
• • •
• •
I wasn't supposed to go out in the sun
after my transplant, but I did anyway. The sun aggravated the mild GVHD that I
had and I turned blotchy. I had itchy light and dark patches on my stomach and
face. I had to go back on steroids.
Veno-occlusive
disease (VOD) is a complication that can occur after bone marrow or stem cell
transplantation in which the flow of blood through the liver becomes obstructed.
Children who have had more than one transplant or previous liver problems are
more at risk to develop VOD. It can occur gradually or very quickly. Symptoms of
VOD include jaundice (yellowing of the skin), an enlarged liver, pain in the
upper right abdomen, fluid in the abdomen, and unexplained weight gain.
Treatment includes fluid restriction, diuretics (such as furosemide),
anti-clotting medications, and removal of all but the most essential amino acids
from IV nutrition (hyperalimentation).