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SCD

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SAVIOR SIBLINGS
INTRODUCTION
Sickle cell disease(SCD) is a group of inherited red blood cell diseases. Sickle cell
disease was first described in 1910 in a dental student with pulmonary symptoms.
Herrick coined the term "sickle" to describe the unusual appearance of his patient's
red blood cells(Figure 1.0). Healthy red blood cells are round and move through small
blood vessels to carry oxygen to all parts of the body. However, the patient who with
SCD, red blood cells will become hard and sticky. The sickle cell dies prematurely,
leading to a persistent shortage of red blood cells. This will lead to pain and other
serious problems. For example, acute chest syndrome and stroke. The advanced and
appropriate stem cell therapy provide for the patient to cure the SCD is called “savior
siblings’. It refers to the child provide an organ or cell transplant for his or her sibling
patient who has the disease difficult to cure, cancer or anemia, stem cell
transplantation can best be treated.
Figure 1.0
EPIDEMIOLOGY
Sickle cell disease affects millions of people worldwide. It’s particularly common
among those the ancestors from sub-Saharan Africa; Spanish-speaking areas of the
Western Hemisphere (South America, the Caribbean, and Central America); Saudi
Arabia; India; Turkey, Greece, Italy and other Mediterranean countries.
It is estimated that:
SCD affects about 100,000 Americans.
SCD occurs in about 1 in every 365 black or African-American babies.
SCD occurs in about 1 in every 16,300 Hispanic births.
Figure 1.1
About 1 in 13 black or African American babies is born with sickle cell trait .
BIOLOGICAL MECHANISM
Patients with sickle cell disease are at increased risk of clinically obvious
depressive symptoms due to chronic stress caused by multiple disease factors. SCD
complications may increase this risk by interacting with the biological processes
behind stress and depression. It is now understood that the potential emergence of
psychopathology depends on the unique interaction between the pressure in the
environment and a person's vulnerability to mental illness.Depressive mood disorders
often co-occur with SCD. The prevalence of clinical depression in children with 8 -14 SCD varied from 25% to 56% from one study to another. Among adults, rates of
depression were similar to those of other chronic diseases, but ranged from 18 percent
to 44 percent, far higher than the 6.8 percent to 7.5 percent reported by African
Americans in the general population. Complications of the disease (including fatigue,
neurological problems, growth retardation and recurrent pain), combined with
multiple stress or such as high frequency of hospitalization and low socioeconomic
status, have been associated with depression in depressed patients. SCD. However,
the main causal direction of these effects is uncertain, as depression can be predicted,
occur with or contribute to these complications.
Characteristics
Female, n (%)
Age, y, mean (SD)
SCD with depression* (n = 50) SCD without depression† (n = 92)
P
36 (72.0)
45 (48.9)
<.01
35.4 (11.8)
33.7 (13.4)
.44
Age group, y, n (%)
.98
18-34
28 (56.0)
51 (55.4)
—
35-49
15 (30.0)
29 (31.5)
—
≥50
7 (14.0)
12 (13.0)
—
42.4 (72.7)
31.9 (40.0)
.27
BMI, kg/m2, mean (SD)
SCD diagnosis, n (%)
.18
SS
28 (56.0)
63 (68.5)
—
1 (2.0)
5 (5.4)
—
SC
15 (30.0)
20 (21.7)
—
Sβ+ thalassemia
5 (10.0)
4 (4.4)
—
SOArab
1 (2.0)
0 (0.0)
—
Sβ0 thalassemia
Severity score, n (%)
.93
0
11 (22)
23 (25)
—
1
18 (36)
36 (39)
—
2
11 (22)
20 (22)
—
3
7 (14)
9 (10)
—
4
3 (6)
4 (4)
—
Insurance, n (%)
.75
Medicaid
19 (44.2)
41 (52.6)
—
Medicare
10 (23.3)
14 (18.0)
—
Private
7 (16.3)
13 (16.7)
—
No insurance/self-pay
2 (4.7)
5 (6.4)
—
Other
5 (11.6)
5 (6.4)
—
SOLUTION
Table 1.2
The savior siblings can be used for any disease that is treated with solution through
hematopoietic stem cell transplantation. It is targeted at genetic testing (mainly effective
single gene) for diseases such as fanconi anemia, congenital pure anemia and
thalassemia, in which the fattening brother can be selected as the savior of the brother
who did not inherit the disease. The procedure can also be used for children with
leukaemia, in which HLA matching is the only requirement and does not rule out any
other obvious genetic disease.Multiple embryos are created and preimplantation genetic
diagnosis is used to detect and select those embryos that are free of genetic diseases and
hLA-matched to existing siblings requiring transplantation. At birth, cord blood is used
for hematopoietic stem cell transplantation.
Figure1.3
By the way,Messianic siblings will feel that the only reason they were brought into this world
is to serve as spare bodies for their siblings, not because their parents wanted another child. This
sense of being used will mock them for life, because they were born "on purpose" in this
they
get older they may feel unloved or unwanted, especially if their siblings die, as they will be
useless. If they can no longer achieve their goals, then what's the point of living? Throughout
their lives, they had been donating to their siblings and doing what they were supposed to do,
and when that was no longer possible, their lives seemed hopeless.
ETHICS&ONE WORLD PROBLEM
Is it ethical for parents to create messianic siblings? Some argue that parental intent plays a
role in considering whether it is ethical to create a messianic sibling. If parents do not intend to
have any more children and they only have messianic siblings for the sake of older children,
then there is a fear of using messianic siblings as a means to an end. If the parents plan to have
more children, then some claim that the messianic sibling was created for her/his own sake and
not just for one purpose. The factors from environmental also cause this problem. We often use
people as a means to an end, but we don't use them entirely as ends, and that's a significant
difference. People often have a second child for the benefit of the first child: they think it's
important for the first child to have a sibling as a playmate, to bring the family closer together
and so on. Even if the messianic sibling is well loved, there is concern that the child may always
feel like a means, at least to some extent. Some believe that the savior's siblings will feel special
or even heroic for the role they play in the family, while others worry that they will be
psychologically hurt because they always feel like a second fiddle or succedaneum. Possible
exploitation of children, such as the potential adverse psychological effects on children born not
for themselves but to save others, and the possible emotional reaction of savior siblings upon
discovering they were born alone to save the recipient's life, rather than being "wanted" for
reasons other than transplantation.
From the world's point of view, obviously, it’s not ethical to create the savior siblings. Their
existence based on the purpose and utilitarianism. They are meaningless after they provide the
organs to their siblings. This is antithetical to philosophy. We could resist more and more savior
siblings become the tool or the “medium” to save others.
Reference List:
Haywood C., Jr (2013). Disrespectful care in the treatment of sickle cell disease
requires more than ethics consultation. The American journal of bioethics :
AJOB, 13(4), 12–14. https://doi.org/10.1080/15265161.2013.768857
Adam, S. S., Flahiff, C. M., Kamble, S., Telen, M. J., Reed, S. D., & De Castro, L. M.
(2017). Depression, quality of life, and medical resource utilization in sickle cell
disease. Blood advances, 1(23), 1983–1992.
https://doi.org/10.1182/bloodadvances.2017006940
Frenette, P. S., & Atweh, G. F. (2007). Sickle cell disease: old discoveries, new
concepts, and future promise. The Journal of clinical investigation, 117(4), 850–858.
https://doi.org/10.1172/JCI30920
Strong, K., Kerridge, I., & Little, M. (2014). Savior siblings, parenting and the moral
valorization of children. Bioethics, 28(4), 187-193.
Trujillo, J. P., & Surralles, J. (2015). Savior siblings and Fanconi anemia: analysis
of success rates from the family’s perspective. Genetics in Medicine, 17(11),
935-938.
Ohene-Frempong, K., Weiner, S. J., Sleeper, L. A., Miller, S. T., Embury, S., Moohr, J.
W., ... & Sickle Cell Disease, T. C. S. O. (1998). Cerebrovascular accidents in sickle
cell disease: rates and risk factors. Blood, The Journal of the American Society of
Hematology, 91(1), 288-294.
Bunn, H. F. (1997). Pathogenesis and treatment of sickle cell disease. New England
Journal of Medicine, 337(11), 762-769.
Steinberg, M. H. (1999). Management of sickle cell disease. New England Journal of
Medicine, 340(13), 1021-1030.
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