By Dr. Turner, Professor Faculty of Medicine & Dentistry
“CRISPR-Cas 9 and Sickle Cell Anemia”
Would you say that gene edited using CRISPR is a lower risk than stem cell transplants?
Gene-editing with CRISPR-Cas9 uses a process similar to stem cell transplants. IMO, it is safer because the stem cell used are from the “patient” thus will not have a risk of GVHD as would stem cells from a sibling. To do the transplant, chemotherapy has to be given in both types and that has definite risks.
How effective is CRISPR in patients who have their vital organs already affected?
Sickle cell patients who have organ damage are at no additional risk from the CRISPR technology but they may not be well enough to undergo the chemotherapy treatment.
I’m getting confused with the different protocols. Does CRISPR use HIV as the lentivector?
Gene-editing with CRISPR-Cas9 does not involve a lentivector such as HIV. That is a different approach which will likely be supplanted by CRISPR.
How soon do you think this treatment will be available to patients in Canada?
Biba Tinga indicated that there may be a trial of CRISPR gene-editing in Toronto within a year or so but I do not have any details on this. The CRISPR-Cas9 technology is controlled by a company associated with Harvard University and one may expect that there will be a significant cost to it.
“21st Century Nursing and the Sickle Cell Patient” By Trudy Sale,
RN BScN Southern Alberta Rare Blood & Bleeding Disorders Comprehensive Care Program
From your experience what would you say are the biggest barriers and myths about using Hydroxy Urea?
We just completed a survey at our clinic asking this same questions. Out of 47 patients that responded to the survey, 17 patients were not on HU. Of the 17 that were not, most had side effects and cancer risk as the major factor for not being on it. We know that there are some side effects that an individual can experience, however we do monitor for side effects and can adjust the dose if necessary.
Does your team ever collaborate with the U of A counterpart?
We do met on occasion (during national and local meetings) and we do collaborate on occasion as well. I certainly think there is room for improvement in this area, and would love to see more collaboration in the future.
How does Hydroxy Urea affect already damaged kidneys?
Unfortunately HU is not able to fix any damage that has been already caused by sickle cell. However, if taken as prescribed it can help slow down or stop organ damage from occurring.
What do you recommend for patients during Covid?
I would recommend that you follow all the provincial and federal guidelines set out by both the government and medical leads. This included physical distancing, frequent hand washing, masking when in public space. It’s important to also try to avoid any triggers for sickle cell pain crisis.
I know the Calgary clinic now offers wallet size emergency cards for SCD patients to carry and present to emergency rooms when they present with a crisis. Is this something practiced across the country? If not, are there plans for widespread adoptions?
Yes this is something that is available through all hemoglobinopathy clinic.
Any tips for sending children back to school or do you recommend home-schooling?
This is really a tough questions with no right or wrong answer. I would say you have to do what you feel is most comfortable for you and what works for your family.
Is an individualized patient pain treatment protocol avail at all ER across Alberta?
No unfortunately not at this time. It is only available to centers that use the electronic chart system that is used by us at the Foothills. This would include all major hospitals in Calgary only. Alberta Health Services is working on a new program that will be used across all major centers on Alberta, but that is a few years away.
Can your service only be accessed by referrals from family physicians?
I am thinking about educational support. Yes we are a referral only service. We are happy to provide education to anyone that requires it.
Is there any correlation between the consumption of foods like milk and development of pain crisis?
Since noticing episodes of muscle pain, cramping, muscle fatigue, and fever resulted in hospital visit following the child’s ingestion of large amounts of milk. It’s now been almost half a year without any episodes of pain or fever. There is no correlation noted in the literature. If someone is lactose intolerant, it stands to reason that milk products might cause pain/ stress/ GI issues which could lead to a pain crisis.
With a pain crisis is there ever analysis specific to change in lactic acid level comparative to lactic acid level during a non-episode blood test?
I checked with the team on this one, as I did not know the answer and no one hasn’t seen anything re: lactic acid levels. If we do them in our patients, they usually are normal during a crisis but really we don’t do them very often.
“Highlights from National: New Drugs and New Outreach Programs”
By Biba Tinga, President of Sickle Cell Disease Association of Canada (SCDAC)
“Blood, You Have It to Give: Meech’s PintSize App”
By Dillon Jayachandran, Arnold Gihozo, Papa-Ollenu Adjaya, and Felix Ikokwu
Extremely cold weather can trigger a pain crisis in a child. Even when bundled up in a snowsuit, the shift in temperature may be enough to cause red blood cells to begin sickling in just a few minutes of outdoor play.