We know that people with Down syndrome (DS) are more susceptible to developing various forms of leukemias, have an increased risk of treatment-related death, and are more prone to relapse.
This population tends to have dysregulated immune systems from birth, making them more prone to autoimmune disorders and blood cancers. The extra chromosome in their DNA triggers additional immune processes and responses.
For those with DS who do develop cancer, their bodies aren’t able to metabolize chemotherapy and other targeted medications as efficiently as non-DS patients, so treatment doses are reduced, and in-patient monitoring is required.
Currently, there is no specific treatment protocol for DS-ALL (Down syndrome and acute lymphoblastic leukemia). Therefore, these patients have extended hospital time, which puts additional and potentially unnecessary strain on patients and their families.
Drug Toxicity in DS
The chromosomal differences between those with DS and other patients with ALL are the exact reason why a standard cancer treatment protocol doesn’t work for them. Due to these DNA differences, people with DS are extra sensitive to and slower to metabolize chemotherapy and other cancer treatment drugs, which also increases their risks for seizures, infections, severe sunburn, and other potentially severe or life-threatening complications.
The Global Down Syndrome Association quoted insights from Dr. John Cripinno, a leading physician of leukemia in patients with DS, who believes there is “a desperate need for less toxic and more effective therapies.“ In the article, Dr. Cripinno explained, “Patients with Down syndrome often react poorly to standard chemotherapy for B-ALL, and their ‘toxicity profile’ means more side effects that can result in extreme loss of red and white blood cells and platelets, inflammation in the digestive tract, and even heart failure. These patients need modified treatment protocols and universal supportive care guidelines that are optimal.”
Extended Hospitalizations
Many pediatric cancer patients can leave the hospital to recover at home after their chemotherapy and medication infusions- while those with DS are considered high risk for complications due to their decreased ability to metabolize drugs- and discharge is delayed while they remain in the hospital for “just in case” observation.
This standard protocol can become frustrating for families who know they can rest better and have a better quality of life at home. Unfortunately, insufficient research has been done to create a specific therapeutic and safe treatment protocol for DS-ALL patients.
What Changes Would the DS-ALL Community Like to See?
The DS-ALL community would like to see additional research completed to develop a treatment protocol for cancer, not just reducing drug dosages. Much more funding and research are also needed to achieve this goal of optional treatment while reducing side effects.
Thankfully, not all DS patients have post-infusion, life-threatening complications. This unique community also hopes that with additional research, a modified chemotherapy and medication protocol can be achieved to allow stable patients to get the treatment they need in a parallel manner as their typical peers and get home faster.
Enter Team Emily
Team Emily exists “to enhance the lives of individuals with Down syndrome who have a leukemia diagnosis by encouraging the funding for research of treatment protocol, as well as providing assistance and resources to patients and their families during their cancer journey.
Heather Pulidio, founder of Team Emily, is clear on their purpose. “We believe that more research should be done to change the treatment protocol to make it safer for patients with Down syndrome so that they are not left in the hospital longer and can have a better quality of life during their course of treatment than their typical counterparts. We will advocate for increased funding to make this happen.”
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