You can live a full, active life when you have Sickle Cell Disease.  You can take part in most of the same activities as other people.  Making smart choices is important in keeping a minor illness from flaring into a crisis.

Review the information below on how to ensure you remain healthy while living with Sickle Cell Disease.

This information is not intended to be medical advice, please consult a health professional or doctor before making any lifestyle changes.

Tips on Living a Healthy life with Sickle Cell Disease

Water and Nutrition

The Importance of Water

To help prevent pain crises, it is important to drink plenty of water throughout the day. This is because dehydration of red blood cells may lead to the sickling process. Adults should drink between 8 to 10 glasses of water (about 4L) daily [1].

Rather than drinking large quantities of water a few times a day, it is better to drink small amounts frequently throughout the day.

Remember that being exposed to the sun for extended periods of time can lead to a higher than normal need for water intake. Be sure to drink extra water in this situation.

Things to Avoid

Sodium: Try to avoid eating foods with high sodium content [1]. This is advised because high sodium levels in your bloodstream will draw water out of your blood cells. Again, this dehydration of your blood causes the hemoglobin to be more concentrated within your blood cells. Being in closer contact with one another makes it more likely for the hemoglobin to clump together and cause cell sickling [2].

Diuretics: Try to avoid drinking diuretics such as caffeinated or alcoholic beverages, as diuretics increase water loss through your urine. If you decide to drink such beverages, do so sensibly and remember that that it will be necessary to increase the amount of water that you are drinking during this time, to make up for the extra water loss that will occur [8]

Folic Acid and Zinc

Folic Acid: Your doctor may recommend a daily supplement of Folic Acid, since your body always requires this vitamin to produce red blood cells [3,4].  Leafy vegetables are a good source of folic acid, however, cooking them will reduce the amount of this vitamin [5].

In addition to helping your body produce red blood cells, daily folic acid supplement has been shown to reduce the incidence of certain bacterial infections [6].

Zinc: Sickle cell disease patients are often deficient in Zinc [7]; a daily supplement of Zinc is shown to significantly improve growth in young patients and also reduce chances of infection [7].

Healthcare and Healthy Living

Routine Dental Care

Regular dental check-ups are important to prevent oral infections that can lead to crisis and complications [14].  If you are undergoing a dental procedure that requires general anaesthesia, this may require hospitalization. Make sure that your dentist is aware of your condition, and the special attention that needs to be given to someone with sickle cell disease who undergoes anaesthesia [15].

Routine Eye Exams

Those with sickle cell disease are often prone to eye damage, leading to permanent vision problems. It is important to have your eyes examined by an eye doctor on a routine basis [15]. If your child has sickle cell disease, it is especially important to ensure that their eyes are routinely examined by a specialist [21].

Routine Antibiotics and Vaccinations

  • Adults should be vaccinated for the flu each year. As well, in addition to all standard vaccinations, it is important to receive the Pneumococcal vaccine (which helps prevent Pneumonia).
  • Children should receive a daily dose of Penicillin from the age of 2 months to at least 5 years of age [15].

Medications to Avoid

  • It is advisable to avoid taking decongestants (e.g., Pseudoepherine). These can cause your blood vessels to tighten and may lead to a pain crisis.
  • As well, all Diuretics should be avoided (substances which increase water loss through urine). Diuretics can be prescribed as medications (e.g., “Diruri”, “Clorpres”, Capaozide”) or can be found in everyday foods, such as: tea, coffee, and pop).
  • To manage pain, use of Acetaminophen or Nonsteroidal anti-inflammatory drugs (NSAIDs) are usually recommended. If pain persists with the use of these, it is recommended to seek medical attention immediately. [8]

Recognizing Early Signs of Complications

In children with sickle cell disease, It is not uncommon for a condition known as Acute Splenic Sequestration to happen, in which blood flowing into the spleen will have difficulty flowing out of the spleen. This causes the spleen to become enlarged as it fills with blood, while the rest of the body does not receive enough supply of red blood cells (leading to Anemia) [16]. Parents or guardians should learn to recognize signs of an enlarged spleen. Family members should learn how to palpate the area of the abdomen where the spleen is located to determine if it is enlarged [16]. Ask your doctor how this procedure is done (It is not complicated). If the spleen becomes enlarged in your child, they may describe pain in the left side of the belly (where the spleen is located).

The anemia that results from this may cause dizziness, fatigue, and the inner eyelids of the child may become pale (known as ‘conjunctivial pallor’) [16]. These signs should always be watched for. If any of them are noticed, call right away for hospital admission.

It has been shown several times that early admission to sickle cell clinic at signs of an enlarged spleen reduces the chances of serious complication later on [17,18].

If a fever of 38 degrees Celsius is reached, this also calls for hospital admission [8].

Hand Washing

Washing your hands can be one of the most important ways of protecting yourself against getting an infection.  Your hands should be washed frequently throughout the day with either soap and water or with gel hand cleaners containing alcohol.

Wash your hands thoroughly before you prepare food for yourself and before you eat food. Wash hands after using the washroom, touching uncooked food (esp. raw meat), touching animals, taking out the garbage, etc).

Preventing Lower Limb Ulcerations

  • Wear ‘compression garments’, proper socks and shoes.
  • Take special care of healing scars (cover them in bandages and socks).
  • Lying down with the ulcered leg elevated can reduce the swelling and lower the pain of an ulcer.

Pregnancy

If you have SCD or SCT and are pregnant or planning to be, it is important to receive early prenatal care and fetal diagnosis to learn to ensure a healthy pregnancy.

Exercise

Ensure that you drink extra water during strenuous exercise and avoid pushing yourself past the point of exhaustion [16]. Understand that with Sickle cell disease, pushing yourself physically past your limits can be dangerous and result in a pain crisis [16].

As with air travel, be mindful of and try to avoid any situations or environments where there is a lower than normal level of oxygen.  Scuba diving and mountain climbing are examples of such situations. As well, make sure that if you are undergoing any procedure in which you are under general anaesthetic (such as dental work), that the healthcare worker is fully aware of the details of your condition.  General anaesthesia can be very dangerous to a person with sickle cell disease if the proper precautions are not taken by the anesthesiologist[15].

Smoking and Second-Hand Smoke

It is important to abstain from smoking. If you are already a smoker, you are encouraged to quit the habit as soon as possible. Cigarette smoking lowers the amount of oxygen in your bloodstream and has been shown to be linked to the occurrence of Acute Chest Syndrome (a painful crisis) in those with Sickle Cell Disease [19, 20].

Travelling with Sickle Cell

When travelling, it is advisable to consult with your physician beforehand.  It is encouraged to carry with you, your medical information, including current medications, and the contact information of your physician. If you are traveling via airplane, It is important to inform the airline of your condition prior to traveling, so that they can prepare for any accommodations that may be necessary (e.g., being provided with extra oxygen during the flight).

It is important to try to avoid being in places with extreme temperatures. Avoid swimming in cold water. Wear warm clothing in cold weather (and even in air-conditioned rooms, in some cases).

Avoid airplanes with non-pressurized cabins. If you are travelling by airplane, it may be necessary to request that you receive extra oxygen.

Check with your airline in advance to know if you are permitted to carry oxygen with you on board the airplane. If you are not permitted to do so, you will need to bring with you and familiarize yourself with a Portable Oxygen Concentrator (POC). A POC is a device that purifies oxygen from the air around you, allowing for this oxygen to be breathed in at times when your oxygen supply is low. It is similar to a Home Oxygen Concentrator, yet much smaller (see below).

Using Oxygen on Airplanes

Check with your airline at least 48 hours before your flight to see if they permit you to take a medical oxygen tank on board the airplane. If it is permitted, you will need to know which models are permitted. The airline may provide the option of renting a tank for the duration of your trip, through a supplier.

Alternatively, the airline may require that you bring your own Portable Oxygen Concentrator (POC), instead of a tank. You will require a note from your physician, prescribing the use of the POC on the airplane [9].

Air Canada Airline and West Jet both permit the use of POCs on their aircrafts.  Each airline, however, has specific regulations on the use of POCs, including the make and model of the device.

To view information on Air Canada’s policies on using POCs on their aircrafts, click here.

Air Canada also requires you to have a ‘medical approval’ form, in order for you to carry a POC with you on their aircrafts. For information regarding how to attain medical approval from Air Canada, click here.

West Jet Airlines also allows passengers on domestic flights (within Canada) to bring a POC or oxygen tanks on board the aircraft, although, certain conditions do need to be met. To view information on West Jet’s policies on carrying an oxygen tank with you on a domestic flight, click here.

Approved Oxygen Concentrators for Airplane Use

(For more information on POC’s that are commonly approved for use on aircrafts, click here.

Airlines that Allow Concentrators

Healthy Living information provided by Daniel Froimovitch.

References:
  1. Fowler, et al: Dietary water and sodium intake of children and adolescents with sickle cell anemia. Journal of Pediatric Hematology/Oncology:  2010 Jul;32(5):350-3.
  2. Brugnara, Carlo: Departments of Pathology and Laboratory Medicine, Harvard Medical School, Boston, Massachusetts
  3. Steinber M.H., sickle Cell Disease and Associated hemoglobinopathies: Cecil Medicine, 23’rd   Ed., 2008.
  4. Zittoun J. Anemias due to disorder of folate, vitamin B12 and transcobalamin metabolism. La Revue du Praticien: 1993;43:1358-63.
  5. Office of Dietary Supplements: National Institute of Health
  6. Prasad, et al:  Effect of zinc supplementation on incidence of infections and hospital admissions in sickle cell disease: American Journal of Hematology: 1999;61:192-02.
  7. Prasad, et al: Zinc deficiency in patients with sickle cell disease: American Journal of Clinical Nutrition, Vol. 75, No. 2, 181-182, 2002.
  8. National Heart Lung and Blood Institute
  9. The Lung Association
  10. Air Canada (Official Website)
  11. West Jet (Official Website)
  12. Federal Aviation Administration (Official Website)
  13. National Home Oxygen Patients Association
  14. Rada, et al: Sickle cell crisis precipitated by periodontal infection: report of two cases. Journal of American Dental Association: 1987 Jun;114(6):799-801.
  15. Harland and Golberg, 2000: Information Centre for Sickle Cell and Thalassemic Disorder
  16. Sergeant G. Sickle cell disease. Oxford: Oxford University Press 1985.
  17. Edmond, et al:  ‘Acute splenic sequestration in homozygous sickle cell disease: Natural history and management’: The Journal of Pediatrics, 1985.
  18. Powell, et al: Acute splenic sequestration crisis in sickle cell disease: Early detection and treatment, Journal of Pediatric Surgery, 1992.
  19. Young, et al: Smoking is a factor in causing acute chest syndrome in sickle cell anemia. J Natl Med Assoc; 84(23):267-271, 1992.
  20. Cohen, et al: Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease: Blood Journal, 115 (18)3852-3854 , 2010.
  21. American Academy of Pediatrics, et al: Policy statement: Eye examination in infants, children, and young adults by pediatricians. Pediatrics, 111(4): 902–907, 2003.