Source: Sickle cell disease.pptx (14 slides) Author: Natasha Roberts Learning Level: Primarily Foundation/Intermediate
1. Definition and Pathophysiology
What Is Sickle Cell Disease?
- Sickle cell disease is the name for a group of inherited health conditions that affect the red blood cells
- The most serious type is called sickle cell anaemia
- Common in people with an African or Caribbean heritage
- Patients produce unusually shaped red blood cells that:
- Do not live as long as healthy blood cells
- Can block blood vessels
Learning Level: Foundation
2. Genetics / Inheritance
| Aspect | Detail |
|---|---|
| Inheritance pattern | Autosomal recessive genetic disease |
| Carrier parents | Both parents are carriers; 1 in 4 chance of inheriting the condition |
| Screening | Diagnosed through newborn heel prick test |
| Most common type | Homozygous sickle cell anaemia (HbSS) is the most common and most severe form of sickle disease in the UK |
| Prevalence | HbSS accounts for about 70% of patients |
Learning Level: Foundation
3. Clinical Presentation / Signs and Symptoms
Sickle Cell Crisis (SCC)
Flare-ups of sickle cell disease result in a sickle cell crisis (SCC):
- Painful episodes which can be very severe and last for days or weeks
- Increased risk of serious infections
- Anaemia which can cause tiredness and shortness of breath
- Delayed growth
- Strokes
- Lung problems
Learning Level: Foundation
SCD and Pain — Mechanism
| Aspect | Detail |
|---|---|
| Mechanism | Vaso-occlusion within the bone marrow vasculature |
| Result | Leads to bone infarction |
| Pathways | Release of inflammatory mediators that activate afferent nerve fibres |
| Character of pain | Throbbing, sharp or gnawing pain |
Learning Level: Intermediate
4. Medical Management
Treatment for SCD
Under haematology team management:
| Treatment | Detail |
|---|---|
| Analgesia | Pain management is central to SCD care |
| Oxygen | Where indicated |
| Hydration | IV and/or oral fluids |
| Blood transfusion | On PICU when required |
Learning Level: Foundation
5. Physiotherapy Assessment and Interventions
Referral Criteria
Physiotherapy is referred when there is a “chest crisis” — defined by the presence of:
- Lower respiratory tract infection (LRTI)
- Chest pain
- Hypoxaemia
Physiotherapy Management
| Intervention | Detail |
|---|---|
| Analgesia | Ensure adequate analgesia before treatment |
| Oxygen | Ensure adequate FiO2 as required |
| Incentive spirometry | Primary physiotherapy intervention |
| Airway clearance techniques | As indicated |
| Supported cough | Assist effective expectoration |
| Mobilisation | As tolerated |
Learning Level: Foundation/Intermediate
6. Incentive Spirometry (IS)
Rationale
- The incentive spirometer is used as an exerciser for inspiration exercises
- Sustained Maximal Inspiration (SMI) — conscious inhalation breathing exercise which corresponds to sighing and yawning at a physiological level
- Encourages a larger breath on inspiration to help improve lung volumes and force air, via collateral channels, behind secretions
- Helps to mobilise secretions up towards central airways to enable easier expectoration by coughing
- Prevents atelectasis and promotes speedier recovery for surgical patients (thoracotomies or laparotomies)
How It Works
- Provides visual and positive feedback when patients inhale at a pre-determined flow-rate or volume and sustain the inflation for a minimum of 3 seconds
- Complete every 2 hours during waking hours
- Support and educate until independent with good technique
- Patient continues IS until crisis ends
Learning Level: Foundation
IS Instructions — Set Up
- Begin by sitting on the edge of your bed or chair
- Hold the incentive spirometer in an upright position
- Breathe out normally
- Place the spirometer mouthpiece in your mouth and close your lips tightly around it
IS Instructions — Technique
- Breathe in through your mouth as slowly and deeply as you can, causing the piston or ball to rise toward the top of the chamber
- Hold your breath for 3—5 seconds or as long as possible, trying to reach the goal indicator
- Remove the mouthpiece from your mouth
- Breathe out normally and the piston or ball will return to the bottom of the chamber
- Rest for a few seconds, then repeat the steps 10 or more times
- Go slowly — take some regular breaths between deep breaths to prevent lightheadedness
- Do this every 1—2 hours when you are awake
- After each set of 10 deep breaths, cough a few times to clear your lungs
Learning Level: Foundation
7. Precautions and Contraindications
Critical Clinical Safety Information
| Precaution | Rationale |
|---|---|
| Manual chest techniques are usually CONTRAINDICATED | High potential for fracture, high likelihood of increased pain, and clotting disorders |
| Care with suction | High likelihood of clotting disorders; relative risks must be clinically reasoned |
| Check blood markers | Always check haematological markers before intervention |
| Discuss with medical team | Collaborative decision-making is essential |
Learning Level: Foundation — This is critical safety knowledge for all physiotherapists
8. Complications / Red Flags
Acute Chest Syndrome (ACS)
Physiotherapy referral is triggered by “chest crisis” which encompasses:
- LRTI in context of SCD
- Chest pain with hypoxaemia
- New pulmonary infiltrate on CXR
General Red Flags
- Worsening pain not responsive to analgesia
- Increasing oxygen requirements
- Signs of stroke (particularly in paediatric SCD)
- Acute drop in haemoglobin
- Splenic sequestration (abdominal pain, splenomegaly)
- Aplastic crisis
Learning Level: Intermediate
9. References
- NHS UK — Sickle Cell Disease overview
- NICE Guidelines — Sickle Cell Disease management (referenced in presentation)
- British Society for Haematology guidelines