The
Procedure
It's called hematopoietic stem-cell transplantation
(HSCT). The words "stem-cell" and "transplant" are a bit
misleading. This is not the type of regenerative treatment that we all read
about in the news nor is Rachelle getting cells from a donor.
The positive results from this treatment are produced by using chemotherapy to
very strongly suppress Rachelle's immune system. The objective is to kill the
components of Rachelle's immune system that have the "memory" to
target her myelin. By erasing this "immune memory" the hope is to
essentially stop the auto-immune disease process.
Once the immune system is suppressed with chemo, Rachelle will get a
transfusion of her own stem-cells which help facilitate the healing of her
immune system. These stem-cells were collected 2 weeks ago and are being stored
until she is ready for the infusion. Hence, the name "hematopoietic
stem-cell transplantation" which means "getting a transplant of your
own stem-cells".
Is
this the same treatment used for cancer?
That is an excellent question! The answer is yes and no.
Rachelle's treatment is based on the procedure used to treat various for forms
of cancer. These procedures are called bone marrow transplants or stem-cell
transplants. Developed 40 years ago, 50,000 cancer patients per year undergo
these procedures.
For cancer, the goal is to fully kill the entire immune system including the
bone marrow where immune cells are produced. To accomplish this, radiation is
often used in conjunction with a more powerful chemotherapy cocktail. These
protocols are called "myeloablative" and patients often require a
bone marrow transplant from a donor to survive.
Unlike cancer, the protocol used to treat CIDP is less toxic. For CIDP, the
target is only the "immune memory" and trials have shown that this
can be accomplished with a less toxic protocol. Therefore, Rachelle's treatment
is considered "non-myeloablative" (also referred to as
transplant-lite) which is less risky than most cancer protocols.
The
Process
Simply stated, Rachelle is getting chemo. She will
experience many of the side-effects you know about including losing her hair (as you have already seen).
Transplant (3 Weeks): Rachelle will be admitted to
Northwestern hospital and will be inpatient for the entire duration of this
phase.
The transplant starts with "conditioning" which is the process of
suppressing (killing) her immune system with chemo. For 5 days Rachelle will
get infusions of cyclophosphamide and rATG.
After a day of rest, Rachelle's harvested stem-cells are infused back into her.
This is considered the "transplant".
For the next few weeks, Rachelle will remain in isolation in the hospital as we
wait for her immune system to start to recover ("engraftment"). This
is the phase when she is most at risk of infection since she essentially has no
immune system.
Recovery
Once Rachelle's blood levels start to show signs of
recovery she will come home. For the first several months she will need to be
very careful as her immune system will still be compromised. She will avoid
sick people, crowds, be careful of what she eats, etc.
Eventually, her new immune system will strengthen and the risk of infection
subsides. However, it will take as long as a year to fully recover from the
effects of the treatment. During this time she will most likely be tired,
experience some pain and a few other side effects.
Schedule
All of the testing and treatments are done in Chicago at
Northwestern University Hospital. Rachelle's Doctor is Dr Richard Burt and his
department is called DIAD.
June 11 - (approximately) June 27: Transplant
Can
I send something?
That is so nice of you to ask!
It's best to send things to our house as they are very strict about what's
permitted in the hospital isolation ward. For example, you can not send flowers,
balloons, fresh fruit or other perishable food items as they can all pose a
risk of carrying bacteria.
Feel free to email/text us for our home address.
Things to know
CHEMO
Worst part is the Lasix (3X per day) and fluids (24 hours). The
purpose is to flush-out the chemo. We're talking 15 bathroom trips per Lasix
dose.
First dose is at 6AM and the last is 10PM. No chance of sleep for two hours
after.
The Blood Cell Count is the transplant scorecard. It provides
insight into Rachelle's status and progress.
Each morning, around 5AM, blood is drawn to run labs. It's a full work-up but
the primary focus is to count four types of blood cells.
1) White Blood Cells (WBCs): Many different cell types which
together comprise the immune system.
Normal Range: 3.5 - 10.5
Data Significance: Provides a general
measurement of Rachelle's immune strength. However, this is not a critical
metric for monitoring the engraftment process nor guiding infusion decisions.
2) Neutrophils: A type of WBC that are the frontline defenders against
bacteria and fungi.
Normal Range: 1.5 - 8.0
Data Significance: This is the most important metric. Once the
neutrophil count falls below .5, Rachelle is considered
"neutropenic". She is most susceptible to infection and
feels the worst.
Rachelle will start to feel better as her immune system comes back online. The
transfusion of her stem cells on Day 0 helps facilitate this process.
"Engraftment" is accomplished when the Neutrophils climb back above
.5.
3) Platelets: Aid in clotting to prevent excessive bleeding.
Normal Range: 140 - 390
Data Significance: A transfusion of platelets is
administered when the count falls below 20.
4) Red Blood Cells (Hemoglobin): Carry oxygen from the lungs to all the
cells in the body.
Normal Range: 11.6 - 17.5
Data Significance: A transfusion of whole blood is
administered when the count falls below 8.
Expected Blood Count Drop and Recovery
Putting it all together, the Cytoxin (chemo) and rATG (anti
T-lymphocyte) at first cause a slight rise in blood cell counts but then drive
a steep decline.
Counts bottom out around Day +2. Rachelle will be pretty sick at this point.
Once her blood cell counts start to increase she will feel better.
The infusion of her stem cells on Day 0 help facilitate recovery and shorten
the amount of time she is Neutropenic.
Engraftment is accomplished when the Neutrophil count >.5
and occurs around Day +10. This is when Rachelle goes home.
The below graph depicts the process.
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Chart format from George Goss.
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The Count
Note: The naming of days using negative numbers prior to
transplant and positive numbers post transplant is how the medical team tracks
the process.
There are a lot of different infusions - hydration, diuretics,
pre-meds, steroids and chemo. Between all the bathroom visits from the fluids
and changing the bags, it's going to be hard to get much rest.
The PICC line is a thin catheter that runs from
her upper arm to her heart. It's used in the hospital to administer IVs,
fluids, meds, etc., and can remain in place for weeks compared to a standard IV
that lasts 3 days.
The line is about the diameter of spaghetti
compared to the neck vas catheter which was more like a slurpy straw.
