In December 2000, Susan Kiechel began experiencing problems picking up her feet. This was devastating to the former star athlete. After many tests, Susan was diagnosed with Charcot Marie Tooth (CMT). This is a genetically inherited, rare, progressive neurological disease that affects the myelin sheath that wraps around the peripheral nerves that control muscles and causes it to slowly shred apart. Over time it causes the loss of normal function to the lower legs/feet and hands/arms. Within 4 months she required leg braces and forearm crutches to walk. The progression of her disease was much quicker and more severe than expected, so she sought additional care from the CMT clinic at Wayne State in Detroit in July 2001. The specialists there quickly realized something wasn’t right and began further, more extensive testing. Her illness was progressing too quickly and becoming too severe to be explained only by the CMT.
In July 2001, Susan was diagnosed with another rare neurological disease called Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). The CIDP was in addition to the CMT. Susan was now fighting two rare and progressive neurological diseases. CIDP is an autoimmune disease that causes the immune system to think the myelin sheath around the nerves is a foreign body and causes the body’s immune system to aggressively attack it. In most cases, CIDP only affects the limbs, though it generally causes paralysis. However, in 5-10% of cases, CIDP progresses to affect the trunk and becomes a life threatening illness because it will affect the nerves around the vital organs. In December 2002, Susan joined the 5-10% of CIDP patients with a life threatening version of the disease.
To slow the progression of CIDP, Susan began Intravenous immunoglobulin (IVIG) treatments on September 12, 2001. These treatments require a hospital stay every 28 days. Susan was determined to fight to slow the progression of these diseases so her children would have a mother alive to raise them. While the treatments have slowed the progression and kept her alive, they have come at an extremely high price. Each month, the disease is able to make some progress. The IVIG slows the progress but it does not stop it. Complications of IVIG treatment include stripping the body of magnesium, Vitamin D, and iron. To counter this, Susan must receive multiple infusions of magnesium and iron each month along with countless other medications to treat the side effects of the IVIG treatment. The IVIG treatments also induce meningitis, which is the swelling of the membrane surrounding the brain and spinal cord. This swelling threatens Susan life constantly and causes excruciating pain. Each month, Susan’s neurological team walks the line of giving enough treatment to keep her alive but not enough that it does more harm.
Susan has endured 235 monthly treatments. This far surpasses the amount of IVIG treatments any other patient in the world has completed. While the treatments have slowed the progression of the CIDP, they are also damaging her body every month. Susan’s body can not withstand many more treatments. She will die soon unless she secures the life-saving treatment she needs.
Stem Cell Transplant
The treatment Susan needs is called a Autologous haematopoietic stem cell transplant (HSCT). This procedure “reboots” the immune system. This is done by using chemotherapy to stimulate the production of stem cells and promote their release into the blood. These stem cells are collected and stored for later use. The patient undergoes extreme doses of chemotherapy to wipe out the rest of their immune system. Once the old immune system is out of the way, the stem cells are implanted back into the patients blood. The stem cells then rebuild a new immune system.
This article goes into further detail about what stem cells actually are, and the general timeline of a stem cell transplant.
Stem Cell Transplants are quite expensive. Susan was approved for a transplant in July. In October, we learned that insurance would not cover the procedure, and that the cost would be almost $155,000. So, we then started a GoFundMe. Between the GoFundMe donations, our own assets, and direct donations from friends and family, we have now surpassed $100,000. Which is amazing, but Susan cannot start the treatment until all the funding is secured.
Time is of the essence. Since the GoFundMe started, Susan contracted two serious MRSA infections. The first one required her port-a-cath be removed and replaced. Port-a-caths usually sit under the skin in the chest, and are used to administer IV medications. However, because of the damage and scar tissue caused by surgeries and procedures, this port is in Susan’s upper thigh. We have run out of places to put another port if something were to happen with this one.
Susan got another infection almost immediately after the first, this time in her neck. In order to keep her port-a-cath, she agreed to do 7 weeks of IV antibiotics to ensure the MRSA was not in her port and was completely done. Susan completed the antibiotics on January 1st.
It’s clear that Susan’s body cannot take much more of the torture that is her monthly IVIG treatments. Every time she enters the hospital, she risks yet another infection. And without an immune system, even a small infection could grow and spread quickly, and kill her.
HSCT is our only option to save Susan, and give her the chance to get the life back that she lost 18 years ago.