Sickle cell Disease (Homozygous Sickle Cell and Minor Disease) is an inherited blood disorder that is hereditary from the parents to the children, in which hemoglobin is produced. As a result red blood cells’ shape gets distorted and blood cells become sickled-shaped, live for a short time, become welded together, clog the small vessels and create painful vaso-occlusive episodes and other complications.
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In Heterozygous Sickle cell anemia (hemoglobinopathy S carrier), anemia doesn’t occur and the individual has a perfectly normal life without requiring treatment. However, in rare cases during acute hypoxia, a crisis may occur.
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A child can be born with sickle cell anemia when both of the parents are carriers of the pathologic gene ( 25% chance), or if one of the parents has the disease and the other one is a carrier (50% chance).
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The daily life of SCD patients can differ because of the diversity of the disease. So, a person with mild SCD symptoms and the appropriate treatment can have a relatively normal life, but in some cases the complications of the disease can take away the regularity of a person’s life.
SCD patients need to get regular monitoring from a hematologist and/or a pathologist in any of the Mediterranean and Sickle cell Disease Units across the country. Regular testing of bone, eye, heart, live upper and lower abdomen, lung and endocrinological examination are also essential.
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Pregnancy while having SCD is a high risk situation both for the mother and the infant So, it is necessary that the potential birthmother should get informed prior to the conceiving in order to make the right decision. Also, during pregnancy, a SCA mother should be monitored by specialized doctors and nursing staff, in order to eliminate possible complications.
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- • When the fever spikes above 38> C.
- When the pain doesn’t subside with painkillers.
- In case of chest pain or difficulty in breathing.
- During acute headache or convulsions.
- In case of paleness or yellowed skin.
- In case of sudden vision changes.
- In case of feeling faint.
In case of delayed development in children, it is necessary to replace the energy and the protein intake that fall short. Because children and teenagers with SCD have higher energy and protein needs compared to their peers.
Moreover SCD patients usually have vitamin, mineral and trace element deficiencies, such as zinc, magnesium, folic acid, vitamin B6, vitamin E and vitamin D deficiencies.
Given that the sickling of red blood cells rises when the cells are dehydrated, it is essential that the SCD patient always stays hydrated.
The SCA symptoms do not appear during the first months of life because of the existence of the fetal hemoglobin which limits the sickling. Usually the clinical signs occur after 6 months of life and the diagnosis of the disease happens during caging episodes, aplastic episodes, and digitalis or chest syndrome cases.
The factors are environmental (dehydration, cold, heat, sudden temperature change, infections, diet, hypoxia etc.), psychological, socio-economic and also genetic.
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The red blood cells that carry the oxygen have an abnormal form during SCD, so the oxygen gets reduced and that leads to intense fatigue. Staying hydrated, getting sufficient sleep and having a balanced diet help to reduce the sense of fatigue.
According to ΦΕΚ Τεύχος Β 5987/31.12.2018 Αριθμ. Φ. 80100/ 50885/3033, the minimum invalidity rate is 67% for SCD patients . In the Joint Ministerial Order of the Vice-Minister of Work, Social Security and Social Solidarity Affairs, and of the Deputy Minister of Finance, it is clarified that this applies regardless of the application of transfusion. Therefore, for the implementation of ν. 2227/1994 the condition of transfusion is not required to be met in the cases of insured persons suffering from Sickle Cell Anemia (homozygous form) or Minor Sickle Cell Anemia. According with the ΦΕΚ 1560/2018 τεύχος ΄Β Αριθμ. Φ. 80100/οικ. 17630/943 on the subject: Replacement of the Table of diseases characterized as irreversible and for which the duration of disability of the insured is determined indefinitely of Φ.80000/οικ.2/1 (Φ.Ε.Κ.Β’ 7/2018, ΑΔΑ: ΩΣΑΦ465Θ1Ω-ΧΟΕ) of ministerial decision.
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In the event that the person examined by the primary health committee considers that the percentage of disability attributed to him is lower than what should be or than what is determined by the uniform table for determining the percentage of disability, he can submit an objection and request to be examined by a secondary health committee commission.
Indeed, those who suffer from SCD are entitled to full old-age pension, as long as they have fifteen years of full actual pensionable service. Also there is no age discrimination, and it doesn’t matter if the person has transfusions or not. (εγκύκλιος Υπουργείου Εργασίας Σ40/81/1546886/2018. (παρ.3 άρθρο 16 νόμος 2227/1994 ΦΕΚ 129/Α/1994, εγκύκλιος Υπουργείου Εργασίας προς ΕΦΚΑ αρ. πρωτ. Φ80000/61352/1381, προεδρικό διάταγμα 169/2007 άρθρο 1α και 26α ΦΕΚ 210/2007, γνωμοδότηση ΝΣ κράτους 6/2015 περί σύνταξης στην 15ετία στους ασφαλισμένους στον ΟΓΑ).
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Those suffering from congenital hemolytic anemia (Mediterranean, sickle cell, minor sickle cell, etc.) or congenital hemorrhagic disposition (hemophilia, etc.) are granted a monthly financial aid by OPEKA, without income criteria and regardless of whether the sufferer works or not, which does not fall into any category income and is therefore not taxable. In addition, it is not subject to any detention or stamp detention, it is not confiscated, nor is it set off against already confirmed debts to the State, individuals or credit institutions and it is not counted against the income limits for the payment of any social or welfare benefit. ΚΥΑ Γ4/Φ.167/2073/1982 (ΦΕΚ Β 640/31-8-82)
Public employees when they retire are also entitled to the Disability Allowance – Captain Presidential Decree 169 /2007 φεκ 210/2007 τεύχος Ά Άρθρο 54 παρ. 10
According with Άρθρο 5 παρ. 4 Ν.3408/05 (ΦΕΚ 272/Α’/4.11.2005) στο τέλος του άρθρου 54 του Κώδικα Πολιτικών και Στρατιωτικών Συντάξεων προστίθεται παράγραφος ως εξής:
Το επίδομα της παραπάνω παραγράφου 5 δικαιούνται και τα πρόσωπα του προτελευταίου εδαφίου της περ. α της παρ. 1 του άρθρου 1, καθώς και αυτά του τέταρτου εδαφίου της περ. α της παρ. 1 του άρθρου 26.
State pensioners who receive the above allowance are still entitled to the welfare allowance.
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- Exemption from income tax for people with disabilities and invalidity rate 80% and over.
Με τις διατάξεις της περ. ε’ της παρ. 2 του άρθρου 14 του ν.4172/2013 it is stipulated that, among other income from salaried work and pensions, wages, pensions and fixed remuneration granted to disabled people with a disability rate of at least eighty percent are exempt from tax (80%). - ΕΝ.Φ.Ι.Α. 100% discount for PWDs with a disability rate of 80% or more
Με τις διατάξεις παρ.2 του άρθρου 7 του ν. 4223/2013 (ΦΕΚ 287 Α’) it is defined that by an act of the Tax Administration a discount of one hundred percent (100%) is granted on the EN.F.I.A. to the taxpayer, his or her spouse or any of the dependent children of his or her family who has a disability of eighty percent (80%) or more, as long as the other conditions are met cumulatively. - Tax reduction for PWDs with a percentage of 67% or more
According with περ. α’ του άρθρου 17 του ν. 4172/2013, an additional tax reduction of two hundred (200) euros is provided for the taxpayer and his dependents, who are persons with at least sixty-seven percent (67%) disability based on the opinion of the Disability Certification Center (KEPA) or the Superior of the Army Health Service (A.S.Y.E.) for disability certification. Occupational or insurance disability is not taken into account.
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SCD patients are entitled to road tax exemption for a car up to 1650 c.c. Parents of disabled minors or the persons who have guardianship of minors are also entitled to the same exemption from road tax (ΠΟΛ.1127/2018 Κοινοποίηση των διατάξεων του αρ. 29 του ν. 4538/2018 (85 Α’). Αφορά το (άρθρο 16 παράγραφος 1 του ν. 1798/88 (ΦΕΚ 166/Α΄).
Στο ΦΕΚ 2710/Τ.Β/24-10-13 state the conditions for the KEPA so that it is written in the KEPA decision that “it falls under article 16 of Law 1798/88 (ΦΕΚ 166/Α΄) on the exemption from traffic and car registration fees”.
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Yes, candidates that suffer from severe diseases can be admitted to the university without examinations, over 5% of the candidate’s places. 35 του Ν.3794/2009 (ΦΕΚ 156/τ.Α’), as changed με την παρ. 24 του άρθρου 39 του Ν. 4186/2013 (ΦΕΚ 193/τ.Α’), με την παρ. 2 του άρθρου έκτου του Ν. 4218/2013 (ΦΕΚ 268/τ.Α’), με το άρθρο 7 του Ν.4283/2014 (ΦΕΚ 189/τ.Α’), με την παράγραφο 1 του άρθρου 13 του Ν.4452/2017 (ΦΕΚ 17/τ.Α΄), με το άρθρο 108 του Ν.4547/2018 (ΦΕΚ 102/τ.Α΄) καθώς και την με αρ. Φ.153/146145/Α5/20-09-2019 (ΦΕΚ 3557/Β’/2019).
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Despite the fact that currently there is a lack of effective treatment for SCA, there are many ongoing clinical studies that contain many therapeutic agents and medications to aid SCA sufferers.
Treating the disease remains supportive but symptomatic. So for now, there are the following treatment approaches:
- hydroxyurea has been offered with very satisfactory results, for almost the last 30 years, as a therapeutic solution for any patient who has repeated hospitalizations due to acute pain or acute chest syndrome and other events
- the tactic of blood transfusions, and/or blood transfusions and/or blood cell transfusions are used to prevent complications. In particular, red blood cell transfusion may be required as an urgent, life-saving option for the treatment of acute complications
- hematopoietic cell transplantation (HCT) is a potentially curative treatment for sickle cell disease (SCD), but is not without risks. Most blood cell transplantations that are being carried out today to SCA patients, use identical siblings as carriers and they are concerning mainly children and teenagers.
- reduction of inflammation and oxidative stress. Recently, glutamine administration as a dietary supplement has begun. L-Glutamine is an essential amino acid of the body and some research has shown that it helps to deal with oxidative stress in Sickle Cell patients, possibly contributing to fewer painful attacks, more energy and well-being and a better health and therefore quality of life. Additional studies are being carried out about its effectiveness.
- reactivation of fetal hemoglobin (HbF), although it is the main action of hydroxyurea, other ways to achieve the goal are being investigated
- gene therapy, is done by transplanting stem hematopoietic cells. Studies are being carried out and so far, the results are discouraging. Furthermore, it involves high technical requirements and cost.