Our Approach
Dr. Ayer has been a leader in the provision of care of patients with sickle cell disease for over 20 years. She was the first doctor to provide a true medical home for adults with sickle cell in our community. She provides primary care including well and sick visits, management of sickle cell related medications and pain medicines, acute care including outpatient infusions for patients with sickle cell disease. As she has expanded her practice to include integrative medicine care and lifestyle medicine, she is one of the few doctors to provide these services to sickle cell patients.
Conventional Care
Conventional care for sickle cell has been limited with few new prescription sickle cell drugs added in last few years. Here is a list of drugs commonly prescribed for sickle cell disease. We offer all conventional care.
Hydroxyurea: Works by increasing the production of fetal hemoglobin (HbF), a type of hemoglobin typically found in newborns that helps prevent the sickling of red blood cells. Increased production of HbF, reduces HbS polymerization and red blood cell sickling. Makes red blood cells larger and more flexible, and improves blood flow. It also reduces the number of white blood cells and platelets, which are involved in vaso-occlusion. This medication reduces the frequency and severity of pain crises, decreases the need for blood transfusions, reduces the risk of acute chest syndrome, and may help prevent or reverse chronic organ damage. Approved for children and adults. Only drug known to increase life expectance in SCD.
Endari: Active ingredient L-glutamine. Helps sickle red blood cells tolerate increased oxidative stress by restoring the ratio of NADH to NAD total, which are crucial coenzymes for regulating and preventing damage due to SCD. Significantly reduces the frequency of painful sickle cell crises. In studies, Endari reduced the number of days in hospital due to sickle cell pain and reduced occurrence of acute chest syndrome, a serious complication of SCD. It may reduce the occurrence of symptoms of SCD in children above 5 years and adults.
Adakveo: Believed to reduce the frequency of sickle cell crisis in people aged 16 years and older with sickle cell disease. Administered by intravenous (IV) infusion every 4 weeks. In the SUSTAIN study, patients treated with Adakveo experienced a reduction in pain crisis compared to placebo. However, subsequent STAND trial overseas did not confirm the previously observed benefits resulting in pulling the drug off market in Europe. The drug is still available in the US. This drug is often complicated by pain during or after treatment within 24 hours.
Sickle Cell Day Hospital
A Sickle Cell Day Hospital provides specialized outpatient care for individuals with sickle cell to decrease need for ER visits and hospitalizations. In addition to offering traditional management of sickle cell disease with disease modifying agents and traditional pain management, we offer a day hospital in our office 6 days a week from 9 to 4 pm in order to help our patients to stay out of the hospital. We also offer complimentary care including medical supplements and radiofrequency therapy.
Curative Treatments
We do not offer any curative therapy in our practice. These procedures require large amounts of chemotherapy and/or radiation that are usually provide in the hospital after a rigorous screening process. These procedures are only offered at few major cancer centers in each are. We do however refer our patients for these procedures and have seen them successfuly performed. Having a knowledgeble referring doctor can be the key to getting into a transplant centers.
Bone Marrow Transplantation: Also known as hematopoietic stem cell transplantation (HSCT), can be a curative treatment for sickle cell disease in children or adults. It involves replacing the sickle cell bone marrow with healthy bone marrow from a donor. Available for both children and adults with SCD. Requires donor who is either HLA-matched sibling or haplo-identical parent usually. Before the transplant, patients undergo conditioning therapy, using chemotherapy and sometimes radiation. The donor's stem cells are infused into the patient's bloodstream, where they travel to the bone marrow and begin producing healthy red blood cells. Associated with high rate of cure and low rate of mortality related to procedure.
Gene therapy: A newer type of transplant therapy, offering the potential to correct the genetic defect in SCD patient's own stem cells, eliminating the need for a donor. This involves collecting stem cells, modifying them in a lab, and then returning them to the patient, allowing for the production of healthyc hemoglobin and reducing or eliminating sickle cell symptoms. The high cost and limited access to transplantation and gene therapy are major challenges.
Integrative Medicine
Integrative medicine is the form of medical care that combines standard evidence based medicine with complimentary alternative therapies. Integrative medicine for sickle cell disease focuses on a holistic approach to pain management and overall wellness, often incorporating non-pharmacological therapies alongside conventional treatments. This approach aims to empower patients to better cope with chronic pain and manage their condition through a variety of strategies.
Lifestyle Medicine
Lifestyle medicine plays a crucial role in managing sickle cell disease by supporting overall health and reducing complications. Key aspects include proper nutrition, hydration, regular exercise, and stress management. Additionally, avoiding harmful substances like smoking and alcohol can help prevent crises and improve long-term health outcomes.
INDIBA Radiofrequency
Physiotherapy can play a crucial role in managing SCD by addressing various complications and improving overall quality of life. It can help manage pain, improve mobility, and prevent pulmonary complications. Physiotherapy interventions include exercise, breathing techniques, and modalities like massage and aquatic therapy, often alongside medication. We offer radiofrequency therapy, a specific type of physiotherapy. INDIBA radiofrequency, using a specific 448 kHz frequency is a non-invasive treatment that can potentially help manage pain and improve tissue repair in conditions like sickle cell disease. It works by stimulating cellular activity and blood flow, which can reduce inflammation and promote healing. While some studies show promising results in pain relief and tissue regeneration, there are no studies specifically in managing sickle cell disease.
Evenflo Nutriceutical
Examples of Integrative Medicine Strategies For SCD
Mind-Body Therapies
Relaxation techniques, meditation, mindfulness, yoga, and hypnosis can help manage pain and improve coping skills.
Acupuncture
Some studies suggest acupuncture can reduce pain intensity and improve pain interference during both acute and chronic pain episodes.
Massage Therapy
Massage can help reduce pain and promote relaxation.
Physical Therapy
Strengthening and conditioning exercises can improve physical function and reduce the risk of complications.
Psychological Support
Counseling, therapy, and support groups can help patients cope with the psychological impact of SCD.
Dietary and Lifestyle Modifications
A balanced diet, hydration, and avoidance of known triggers can help manage SCD and reduce the frequency of pain crises.
Benefits of Integrative Medicine For SCD
Reduced reliance on pain medications:
Non-pharmacological therapies can help decrease the need for pain relievers.
Improved pain management:
Integrative approaches can help reduce pain intensity and improve pain tolerance.
Enhanced quality of life:
By addressing the physical, psychological, and emotional aspects of SCD, integrative medicine can improve overall well-being.
Increased self-efficacy:
Patients can develop greater confidence in their ability to manage their condition and cope with pain.
Patient-centered care:
Integrative medicine focuses on understanding the individual patient's needs and preferences, leading to more personalized treatment plans.
Compenents of Integrated Care
Physical Activity
Recent studies suggest that low-to-moderate intensity physical activity can be safe and beneficial for individuals with SCD. These studies indicate that exercise can improve muscle function, increase capillary density in muscles, and enhance overall physical capacity without causing SCD crises.
Key findings from research:
Improved Muscle Function and Capillary Density: Studies have shown that regular, low-to-moderate intensity exercise can lead to significant improvements in muscle function and an increase in the density of small blood vessels (capillaries) in muscle tissue, according to the American Society of Hematology.
Increased Exercise Tolerance: Physical activity, especially aerobic exercise like walking, jogging, or cycling at moderate intensity, has been linked to improved cardiovascular fitness and increased tolerance for physical activity.
Reduced Vaso-occlusive Crises: While historically, exercise was often avoided due to concerns about triggering crises, some studies suggest that controlled, moderate-intensity exercise may actually help reduce the frequency of SCD crisis.
Potential for Improved Quality of Life: Exercise can positively impact mood and overall well-being by releasing endorphins and improving blood flow and oxygen delivery to tissues, reducing the risk of vaso-occlusion and tissue ischemia.
Diet
Good nutrition from a well-balanced diet and hydration may reduce complications and improve the patient’s quality of life. Focus on a balanced intake of fruits, vegetables, whole grains, and protein-rich foods, while ensuring sufficient intake of calcium, zinc, and vitamin D. Adequate hydration, especially with water, is vital to prevent red blood cell sickling and potential pain crises.
Key aspects of a sickle cell disease diet:
Hydration: Drink plenty of water, especially when it's hot, when exercising, or during pain episodes. Aim for at least eight 8-ounce glasses daily.
Fruits and Vegetables: Include a variety of colorful fruits and vegetables for essential vitamins and minerals.
Whole Grains: Choose whole grains like brown rice, quinoa, and whole wheat bread for sustained energy.
Protein: Incorporate protein sources like lean meats, poultry, fish, beans, nuts, and seeds. Some studies have found benefits to high protein diet in sickle cell disease.
Limit Sugary Drinks: Reduce intake of sugary drinks like soda and fruit juices
Limit Processed Foods: Reduce processed foods. Foods that come in boxes and packages manufactured in factories like cereal. Meats like bacon and ham.
Maintain Healthy Weight: Both underweight and overweight may be problematic.
Mind-Body Therapies
Mindfulness meditation and other mindfulness techniques can help individuals focus on the present moment and manage pain and stress. Mind-body therapies like yoga, cognitive behavioral therapy (CBT), and guided imagery have shown promise in reducing pain, improving mood, and enhancing coping mechanisms in individuals SCD. These therapies, often used in conjunction with traditional medical approaches, can improve patients' overall well-being and quality of life.
Yoga: Studies have shown that yoga can be an effective tool for pain management and improving mood in individuals with SCD. Studies indicate that yoga combined with mindfulness and breathing exercises, can be beneficial and well-tolerated for individuals with SCD. These practices have shown promise in managing pain, anxiety, and overall well-being in both children and adults with SCD. Studies have shown that yoga interventions can lead to a reduction in pain intensity, especially during sickle cell crises. Yoga may also help reduce anxiety and depression, which are frequently experienced by individuals with SCD and can exacerbate pain.
Cognitive Behavioral Therapy (CBT): CBT is a type of therapy that helps individuals identify and change negative thought patterns and behaviors, has been shown to be effective in managing chronic pain associated with SCD. CBT can reduce pain frequency and intensity, improve pain controllability, and enhance coping skills. CBT has been associated with improved mood, reduced fatigue, and decreased healthcare utilization in individuals with SCD.
Guided Imagery: Guided imagery, a technique that involves using mental images to promote relaxation and reduce pain, has demonstrated positive effects on pain intensity and frequency in individuals with SCD. Some studies have also shown that guided imagery can improve pain coping efficacy and self-efficacy.
Hypnosis: Hypnosis has been shown to decrease pain intensity and improve pain threshold and tolerance in SCD patients.
Acupuncture: Acupuncture has shown promise in improving overall quality of life in patients with SCD, possibly by alleviating pain and its associated effects like anxiety and sleep disturbances. Some preliminary research indicates that acupuncture may help to decrease inflammatory and immune responses, which are implicated in the pain and complications of SCD. Acupuncture can potentially enhance microcirculation, which may help mitigate the blood flow blockages caused by sickle-shaped cells. Acupuncture's calming effects can contribute to improved mental well-being, which is especially beneficial for individuals managing chronic pain and the stress of living with SCD.
Supplements
The following is a list of vitamins and supplements that my be useful in sickle cell disease. Most doctors are not trained in the use of supplements. Each patient to consult a knowledgeable professional for recommendations.
Folic Acid: Essential production of red blood cells. People with SCD have red blood cells that break down faster, and folic acid helps to replace them. Most children with SCD are given a daily folic acid supplement, often 1mg (1000 mcg). Most adults with SCD continue supplementation with folic acid.
Vitamin D: Vital for bone health, immune function, and reducing pain. People with SCD are often deficient in vitamin D and may benefit from supplementation, especially since it can help with bone health and potentially reduce the risk of fractures.
Zinc: Needed for growth, wound healing, immune function and maintaining red blood cell stability. Deficiency in zinc is common in SCD patients and can lead to growth problems, increased pain episodes, and decreased bone density. Good sources include meat, poultry, seafood, beans, nuts, and whole grains.
Vitamin E: Vitamin E is an antioxidant that can help protect red blood cells from damage. It also plays a role in immune function.
Vitamin C: Vitamin C is also an antioxidant and can support the immune system.
Omega-3 fatty acids: These can help reduce inflammation and improve blood flow, which is beneficial for SCD patients.
Magnesium: Magnesium may help prevent red blood cell dehydration and reduce pain crises.
Calcium and Vitamin D: Include calcium-rich foods like milk, yogurt, and cheese, and ensure adequate vitamin D intake, possibly through fortified foods or supplements.
Aloe vera: May help prevent the sickling of cells and reduce pain crises episodes.
Lemongrass: May help increase red blood cell counts.
Garlic: May be effective in treating sickle cell anemia due to its antioxidant effects, preventing the growth of dense cells.
Carica papaya: Extracts, particularly from the leaves and fruit pulp, have shown anti-sickling activity. They may also help protect red blood cell membranes.
Turmeric: Research suggests that turmeric rhizome extract has anti-sickling properties.
Fenugreek: Studies indicate that fenugreek seed extract may also have anti-sickling properties.
4. Avoiding Harmful Substances
Smoking: Smoking worsens lung complications, including acute chest syndrome.
Alcohol: Alcohol can cause dehydration, which is a risk factor for sickle cell crises.
5. Other Important Considerations
Temperature control: Avoid extreme temperatures, both hot and cold, as they can trigger crises.
Altitude: Be cautious at high altitudes, as the lack of oxygen can increase the risk of a crisis.
Mental health care: SCD can affect mental health, so consider seeking professional support.
